Sjögren's syndrome
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Sjögren's Syndrome
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Description
Causes and symptoms
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Sjögren's syndrome
[sha´grenz]Sjögren's syndrome
(shō′grənz, shœ′-) alsoSjögren syndrome
(-grĕn)Sjögren's syndrome
Sjögren's syndrome
An immunological disorder causing reduced secretion of many exocrine glands as a result of damage by antibodies produced by the body (autoantibodies). Affected glands are infiltrated with lymphocytes. Their malfunction causes severe dryness of the eyes, mouth and vagina. The syndrome is commonly associated with other immunological disorders, such as RHEUMATOID ARTHRITIS, SYSTEMIC LUPUS ERYTHEMATOSUS and MYASTHENIA GRAVIS. Treatment is by anti-inflammatory and cytotoxic drugs and symptomatic relief can be obtained from artificial tears, moistening mouth sprays and vaginal lubricants. (Henrik Samuel Conrad Sjögren, b. 1899, Swedish ophthalmologist).Sjögren’s Syndrome
DRG Category: | 546 |
Mean LOS: | 5.2 days |
Description: | MEDICAL: Connective Tissue Disorders With CC |
Sjögren’s syndrome (SS) is the most common autoimmune rheumatic disorder after rheumatoid arthritis (RA). It is a chronic, progressive disease associated with other diseases, such as RA, in approximately 50% of the cases.
SS is characterized by failure of exocrine glands and by diminished tearing and salivary secretion (sicca complex). It results from chronic exocrine gland dysfunction, although the disorder may also involve other organs such as the lung and kidney. SS may be a primary disorder or it may be associated with connective tissue disorders, such as RA, scleroderma, systemic lupus erythematosus, and primary biliary cirrhosis. Tissue damage results either from infiltration by lymphocytes or from the deposition of immune complexes. The overall prognosis for patients with SS is good, and the disease seldom leads to significant complications.
Causes
The direct cause of SS is unknown. It seems likely that both environmental and genetic factors (see Genetic Considerations) contribute to its development. In a genetically susceptible individual, either bacterial or viral infection or exposure to pollen may be the catalyst for SS.
Genetic considerations
SS is believed to be a complex disorder with a genetic susceptibility and an environmental trigger. Associations with the human leukocyte antigen variants DRB1, DRB1∗03, and DQB1∗02 have been described.
Gender, ethnic/racial, and life span considerations
SS occurs mainly in women: 9 out of 10 patients are female. The mean age of occurrence is 50, although it has been reported in children who develop parotid gland enlargement. A small percentage of women who develop SS may have accompanying nonlymphoma and lymphoid malignancies. No racial and ethnic considerations are known.
Global health considerations
SS occurs around the globe with similar prevalence as found in North America.
Assessment
History
Establish a history of either autoimmune or lymphoproliferative disorders. Rule out other causes of oral and ocular dryness; ask about any history of sarcoidosis, endocrine disorders, anxiety or depression, and radiation therapy to the head and neck. Many commonly used medications produce dry mouth as a side effect, so take a thorough history of medications. In patients with salivary gland enlargement and severe lymphoid infiltration, rule out malignancy. Approximately 50% of patients with SS have confirmed RA. When you ask about symptoms, the patient may report gritty or sandy sensations in the eye or a film across the visual field. Patients may also report dryness of the mouth, burning oral discomfort, difficulty in chewing and swallowing dry foods, increased thirst, and reduced taste. The patient may also report the incidence of many dental caries and chronic middle ear infections. Dryness of the vagina and vulva leads to reports of painful urination, itching, and painful or difficult sexual intercourse.
Physical examination
The most common symptoms are dry eyes and dry mouth. The patient’s tongue is often red and dry with atrophic taste buds. Unilateral or bilateral parotid and salivary glands may be hardened and nontender. Dental caries are a common finding. The dryness may make talking difficult. Patients may have a dry, chronic cough and an increased incidence of upper and lower respiratory tract infections, which has resulted in a chronic vocal hoarseness. Nasal mucosa may be dry and reddened. Gastrointestinal tract involvement may lead to gastritis, esophageal mucosal atrophy, and difficulty in swallowing. Genitalia may appear dry and possibly ulcerated. Involvement of the exocrine glands leads to dry, tough, scaly skin; decreased sweat; and chronic itching.
Psychosocial
The patient with SS has complaints that may have been attributed to multiple causes, possibly over years. Because SS is closely related to systemic lupus erythematosus and RA, the patient may have been misdiagnosed, causing considerable emotional distress. Because SS affects senses, such as sight and taste, and also sexuality, assess the patient’s ability to cope with the presenting symptoms and other common complaints.
Diagnostic highlights
Test | Normal Result | Abnormality With Condition | Explanation |
---|---|---|---|
Salivary gland biopsy | Normal salivary gland cells | Presence of inflammatory cells and immune complexes | Identifies abnormal cells in secretory glands and ducts |
Slit-lamp examination | Normal examination | Detection of dryness of conjunctiva and reduced tearing | Identifies reduced tear film and dryness of eyes |
Other Tests: Complete blood count, antinuclear antibodies, rheumatoid factor, anti-alpha-fodrin antibody, erythrocyte sedimentation rate, tear osmolarity, fluorescence clearance test, parotid flow rate, radionuclide scan, Schirmer test (test strip of number 41 Whatman filter paper placed near the lower conjunctival sac to measure tear formation; healthy persons wet 15 mm or more after 5 minutes)
Primary nursing diagnosis
Diagnosis
Impaired skin integrity related to diminished or absent glandular secretionsOutcomes
Tissue integrity: Skin and mucous membranes; Wound healing: Primary intention; Knowledge: Treatment regimen; Self-care: Hygiene; Treatment behavior: IllnessInterventions
Skin and membrane surveillance; Skin care; Medication administration; Fluid balance; SurveillancePlanning and implementation
Collaborative
Care of the patient with SS is designed to treat symptoms. Instill artificial tears as often as every 30 minutes to prevent corneal ulcerations or opacifications that may be caused by insufficient lacrimal secretions.
Patients who also have RA may benefit from a combined program of medical, rehabilitative, and surgical treatments. The primary goals are suppression of further joint and tissue inflammation, maintenance of joint and tissue function, repair of joint damage, and relief of pain.
Pharmacologic highlights
Medication or Drug Class | Dosage | Description | Rationale |
---|---|---|---|
Cyclosporine ophthalmic eye drops | Apply 1% or 2% solution bid | Immunosuppressant | Suppresses T-cell formation and has been used to increase lacrimal gland function in SS |
Other Drugs: Corticosteroids; eye lubricants; omega-3 fish oil; sustained-release cellulose capsules (hydroxypropyl cellulose) may be used. Topical (diquafosol) and systematic (pilocarpine, cevimeline) stimulators of tear secretion may be used. If eye infection develops, the patient receives antibiotics; topical steroids are avoided.
Independent
Suggest the use of sunglasses to protect the patient’s eyes from strong light, wind, and dust. To reduce the risk of infection caused by dry eyes, advise the patient to keep his or her face clean and to avoid rubbing the eyes. Mouth dryness can be relieved by using a swab or spray and by drinking plenty of fluids, especially at mealtime. Sugarless throat lozenges can also relieve mouth dryness without promoting tooth decay. Meticulous oral hygiene should include regular brushing, flossing, and fluoride treatment at home, along with frequent dental checkups. Teach the patient to avoid medications that decrease saliva production, such as atropine derivatives, antihistamines, anticholinergics, and antidepressants. Suggest high-protein, high-calorie liquid supplements to patients with painful mouth lesions. Soft foods may be easier for patients to swallow. Parotid gland enlargement can be treated with local heat and analgesia.
Respiratory dryness can be reduced by using a humidifier at home and at work. Nasal dryness can be relieved by the use of normal saline solution drops. Moisturizing lotions can ease skin dryness, as can avoiding lengthy hot showers or baths. Patients should avoid sunburn and any lengthy exposure to the sun; recommend using a sunscreen when outdoors. Water-soluble lubricating jelly is an effective lubricant during sexual intercourse.
Evidence-Based Practice and Health Policy
Segal, B.M., Pogatchnik, B., Holker, E., Liu, H., Sloan, J., Rhodus, N., & Moser, K.L. (2013). Primary Sjogren’s syndrome: Cognitive symptoms, mood, and cognitive performance. Acta Neurologica Scandinavica, 125(4), 272–278.
- In a study comparing 39 adult patients with primary SS to 17 healthy control participants, 47% of SS patients reported depression compared to 6% of control participants.
- Depression was significantly correlated with cognitive functioning (r, 0.782; p < 0.001). Compared to control participants, patients with SS performed worse in psychomotor processing (p = 0.027) and verbal reasoning (p = 0.007).
Documentation guidelines
- Physical findings of dysphagia (difficulty swallowing)
- Physical findings of presence of red, irritated, or ulcerated mucosal membranes
- Reaction to remoisturizing eyes, mouth, and other affected areas
Discharge and home healthcare guidelines
Instruct the patient to avoid sugar; tobacco; alcohol; and spicy, salty, and highly acidic foods. Recommend high-calorie, protein-rich liquid supplements to patients with painful mouth lesions. Teach the patient how to instill eye drops, ointments, or sustained-release capsules. Advise the patient to avoid over-the-counter medications that include saliva-decreasing compounds, such as antihistamines, antidepressants, anticholinergics, and atropine derivatives.
syndrome
aggregated objective signs, subjective symptoms and specific pathologies that typify specific conditionsacquired immunodeficiency syndrome; AIDS severe reduction in numbers of T4 lymphocyte helper (CD4) cells (due to infection with human immunodeficiency virus [HIV]) and resultant compromise of humoral and cell-mediated immunity; patients show lymphadenopathy, opportunistic infections (e.g. tinea and verrucae) and unusual infections (e.g. histoplasmosis, gastrointestinal tract candidiasis, Pneumocystis carnii pneumonia [PCP]), unusual malignancies (e.g. Kaposi's sarcoma), wasting diseases and presenile dementia
acute compartment syndrome; ACS increased lower-limb intracompartmental pressure on exercise (exercise expands muscles, increases intracompartmental pressures, inducing pain); treated initially by rest, immobilization, non-steroidal anti-inflammatory drugs; severe cases may require surgical decompression (fasciotomy)
anterior tarsal syndrome; ATS deep peroneal nerve entrapment at anterior ankle/dorsal talonavicular joint, due to restriction of ankle dorsiflexion (e.g. tight boots; ski boots), or local soft-tissue trauma (e.g. dorsal tarsal exostoses); characterized by extensor hallucis longus weakness, dorsal foot paraesthesia and numbness of first intermetatarsal space (symptoms can be induced by deep peroneal nerve percussion as crosses the anterior aspect of the ankle joint, or by ankle joint plantarflexion whilst simultaneously dorsiflexing toes)
anterior tibial compartment syndrome ischaemic necrosis of anterior compartment muscle fibres, due to local arterial compression by engorged muscles, after unaccustomed exertion
anterior tibiotalar impingement syndrome anterior ankle pain at ankle dorsiflexion (e.g. at midstance, just before heel lift) due to inferior tibial/neck of talus exostosis
Apert's syndrome type Ia acrocephalosyndactyly, characterized by features of Carpenter's syndrome, with lesser digital (2-5) fusion into one mass, usually with a common mega-nail
Apert-Crouzon syndrome type IIa acrocephalosyndactyly characterized by features of Carpenter's syndrome with additional craniofacial dysostosis, maxillary hypoplasia, and 2-4 digit fusion
Bazex syndrome; acrokeratosis paraneoplastica keratoderma (i.e. erythema, scaling and irritation) of skin of ears, nose, hands and feet and later generalized hyperkeratosis in men with underlying internal malignancy; condition regresses when underlying malignancy is resolved
Behçet's syndrome chronic vasculitic disease of unknown cause; characterized by seronegative arthritis of knees and ankles, elbows and wrists, mouth ulcers, erythema nodosum, visual impairment and cerebrovascular accident
benign familial joint hypermobility syndrome; BFJHS generalized joint hypermobility, diagnosed as 2 major/1 major + 2 minor/4 minor criteria (see Table 1) in the absence of Ehlers-Danlos syndrome, Marfan's syndrome and osteogenesis imperfecta
Brocq-Lyell syndrome; toxic epidermal necrolysis severe, acute, systemic drug reaction characterized by hyperpigmented skin lesions and epidermal detachment
Brown-Séquard syndrome hemiparaplegia and hyperaesthesia, with ipsilateral loss of stereognosis and contralateral hemianaesthesia; due to unilateral spinal cord lesion
carpal tunnel syndrome pain, paraesthesia and loss of power of palmar muscles; associated with rheumatoid arthritis
Carpenter's syndrome; acrocephalopolysyndactyly oxycephaly, bradysyndactyly and polydactyly of the feet, with learning difficulties
Charcot's syndrome see intermittent claudication
chronic compartment syndrome; CCS; chronic exertional compartment syndrome exercise-induced fascial compartment pain; caused by compromised circulation and relative ischaemia of intracompartmental tissues, with long-term muscle and nerve dysfunction and damage; recalcitrant cases require surgical decompression through fasciotomy (see syndrome, acute compartment)
compartment syndrome see syndrome, acute compartment; syndrome, chronic compartment
complex regional pain syndrome; CRPS; chronic regional pain syndrome neuroinflammatory dysfunction, due to ion interaction of nociceptive C-fibre nerve endings, the sympathetic nervous system and spinal cord efferent motor nerves; characterized by vasomotor instability, hyperalgesia and impaired motor function; diagnosed from clinical presentation, symptoms reduction on administration of sympathetic nerve blockade, and intense, focal periarticular uptake of contrast medium in a delayed imaging-phase bone scan; treated by early, aggressive physical therapy to prevent contracture and muscle wasting, symptomatic relief by sympathetic nerve blockade, non-steroidal anti-inflammatory drugs, tricyclic antidepressants and anticonvulsant medication; immobilization is contraindicated
complex regional pain syndrome type 1; CRPS 1; reflex sympathetic dystrophy; Sudek's atrophy; allodynia sympathetic nervous system-mediated acute pain and vasomotor instability, triggered by minor or surgical trauma without obvious nerve injury; affects women more than men; pain is excessive and out of proportion to severity of initiating injury; diagnosis is based on clinical symptoms aided by bone scan, laser Doppler studies and thermography; patients may show anxiety, depression and disturbed sleep; condition is difficult to manage; patients suspected of CRPS 1 should have early referral to a pain clinic (see Table 2); presents in three stages:
stage 1 acute phase, lasting 2-3 months, with regional severe burning pain, warmth and swelling triggered by stress/light touch, bone demineralization, skin trophic changes
stage 2 dystrophic phase/Sudek's atrophy; lasting for several months; characterized by constant unrelenting pain, exacerbated by any stimulus, and tissue cyanosis, coolness and induration, and diffuse osteoporosis
stage 3 atrophic phase, characterized by reduced/absent/intractable pain, irreversible atrophy of skin/subcutaneous tissues, flexion contractures of foot, advanced osteoporosis with a 'ground-glass' appearance on X-ray of affected bone
complex regional pain syndrome, type 2; CRPS 2; causalgia; sympathetic pain syndrome persistent and severe skin paraesthesia/burning sensations; caused by trauma to peripheral sensory nerve fibres; symptoms, progress and treatment are similar to that of CRPS 1
Conn's syndrome primary aldosteronism; characterized by headaches, thirst, nocturia, polyuria, hypovolaemia, fatigue, hypertension, alkalosis, and potassium depletion
constrictive band syndrome intrauterine development of deep, tight, circumferential folds around leg/foot, and compromised limb development distal to band (e.g. autoamputation; marked oedema of distal tissues); thought to relate to strands of amniotic membrane enwrapping the developing limb
Cushing's syndrome raised blood cortisol (e.g. due to pituitary tumour; long-term steroid therapy); characterized by central obesity, moon-like facies, acne, skin striae, hypertension, decreased carbohydrate tolerance and tendency to diabetes, female amenorrhoea and hirsutism
Down's syndrome chromosomal disorder (trisomy 21) characterized by congenital short stature, broad short hands/feet, characteristic facies (pronounced epicanthic skin folds, flat hypoplastic face, short nose, enlarged tongue), transverse palmar crease, very dry skin, learning difficulties; formerly termed mongolism
Edwards' syndrome trisomy 18, with congenital characteristic facies (micrognathia, low-set ears), rocker-bottom feet, severe learning difficulties; affected children often die in early childhood
Ehlers-Danlos syndrome; Ehlers-Danlos diseases I-X hereditary connective tissue disorder characterized by collagen abnormality, marked generalized skin and blood vessel laxity, and joint hypermobility; skin is readily traumatized and heals slowly; see syndrome, hypermobility
Franconi's syndrome a form of anaemia associated with renal tubule dysfunction; adult Franconi's syndrome shows synostosis with osteomalacia, and acquired Franconi's syndrome is associated with multiple myeloma
Giles de la Tourette syndrome motor incoordination characterized by verbal, facial or limbic tics
Gorlin's syndrome multiple naevus-like basal cell carcinomata, causing small pits and depressions of palmar and plantar skin
Guillain-Barré syndrome; acute inflammatory polyneuropathy; acute idiopathic polyneuritis; infectious polyneuritis; postinfective polyneuropathy sudden-onset, acute, postviral polyneuritis; presents as distal pain, muscular weakness/flaccidity, paraesthesia; spreads proximally over 14-21 days; severe cases show spinal nerve involvement, with respiratory failure and limb paralysis (patient will require life support and anticoagulation to prevent deep-vein thrombosis); spontaneous recovery occurs over several weeks/months; some residual neuromotor effects may persist
Haglund's syndrome prominence of posterior superior lateral area of calcaneum, retrocalcaneal bursitis, Achilles tendon thickening and Achilles tendinitis; diagnostic rearfoot radiographic features include positive parallel pitch lines, loss of retrocalcaneal recess (indicating retrocalcaneal bursitis), Achilles tendon thickening, loss of distinct interface between Achilles tendon and pre-Achilles fat pad
heel pain syndrome see heel pain
heel spur syndrome see heel spur
Howel-Evans syndrome familial palmoplantar keratoderma, with increased risk of oesophageal cancer
Hurler's syndrome; lipochondrodystrophy; dysostosis multiplex autosomal-recessive inherited generalized lipid disturbance and mucopolysaccharoidosis, affecting cartilage, bone, skin, subcutaneous tissues, brain, liver and spleen; characterized by short stature, shortness of neck, trunk and digits, kyphosis, reduced joint mobility, learning difficulties, characteristic facies (so-called gargoylism) and visual impairment
hypermobility syndrome; joint hypermobility syndrome disordered collagen (types 1 and 3) structure, with associated decreased tensile strength of skin/structural tissues; characterized by generalized joint hypermobility, easy bruising, impaired healing, increasing incidence of joint/soft-tissue pain, joint dislocation and osteoarthritis; a presenting feature of benign familial joint hypermobility syndrome (BFJHS) (see Table 3), Ehlers-Danlos syndrome, Marfan syndrome and osteogenesis imperfecta
iliotibial band syndrome; ITBS; iliotibial band friction syndrome; ITBFS overuse-associated, friction-induced inflammation of ITB and associated bursa, where ITB moves over lateral femoral condyle (Gerdy's tubercle); due to repeated knee flexion and extension, especially in athletes/cyclists; presents as ITB pain at heel strike progressing to constant ITB pain; early-stage treatment includes a daily stretching programme (see Table 4) and application of heat (pre-exercise) and ice (postexercise) (see Table 5)
joint hypermobility syndrome see syndrome, hypermobility
lobster-claw syndrome extreme form of ectrodactyly; characterized by absence of third and fourth rays
Korsakoff's syndrome confusion and severe memory impairment with confabulation and Wernicke's syndrome, associated with chronic alcoholism
Lyell's syndrome drug-induced, acute skin sensitivity reaction; characterized by acute erythema, urticaria, vasculitis, purpura, marked exfoliation (peeling), flaccid bullae formation, subepidermal separation/detachment
Marfan's syndrome familial, autosomal-dominant, congenital changes in mesodermal and ectodermal tissues; characterized variably by musculoskeletal changes (e.g. increased height, excessive limb length, arachnodactyly; generalized tissue laxity and joint hypermobility), visual effects, and cardiovascular effects (e.g. aortic aneurysm)
medial tibial stress syndrome; MTSS; tibial fasciitis; shin splint muscle fatigue, reduced shock absorption, traction enthesiopathy and periostitis along anterior and posterior medial lower one-third of tibia (see Table 6) secondary to overuse/underpreparation for exercise; exacerbated by exercising on hard surfaces, especially in individuals who pronate excessively; treated by muscle-strengthening exercises, pre-exercise flexibility programme, modification of overall sports exercise programme (see Table 7), in conjunction with gait analysis, orthoses and correct shoe selection
Morquio's syndrome; type IV mucopolysaccharoidosis severe skeletal dysplasia including spine/thorax deformity, irregular epiphyses but normal shaft length of long bones, enlarged joints, flaccid ligaments, waddling gait and urinary abnormalities, due to autosomal-recessive error of mucopolysaccharide metabolism
Morton's syndrome congenital shortening of first metatarsal with apparent shortening of hallux and associated metatarsalgia
Munchausen's syndrome repeated fabrication of illness/symptoms of illness
Munchausen's syndrome by proxy repeated reporting of spurious illness/symptoms of illness by one person about another
musculoskeletal pain syndrome see polymyalgia rheumatica
nail-patella syndrome; hereditary arthrodysplasia autosomal-dominant abnormality of finger/toenails, absent/hypoplastic patella, defects of head of radius and iliac horns, and iris discoloration
nephrotic syndrome peripheral oedema, albuminuria, reduced plasma albumin (hypoalbuminaemia), refractory bodies in urine and raised blood cholesterol
nerve entrapment syndromes local nerve trunk compression (e.g. tibial, medial calcaneal lateral, first lateral branch of calcaneal, lateral plantar, high tibial, popliteal, deep peroneal, superficial, saphenous, sural or medial common hallucal nerves), as in tarsal/carpal tunnel syndromes, plantar digital neuritis, Morton's neuroma; characterized by distressing distal dermatomal sensory (e.g. pain and paraesthesia) and/or motor symptoms (e.g. muscle atrophy) (see Table 8)
Nievergelt-Pearlman syndrome rare autosomal-dominant bone disease causing lower-limb 'rhomboidal' tibia/fibula (crura rhomboidei), joint dysplasias, genu valgum, club foot, deformed toes; more common in males
overlap syndromes see mixed connective tissue diseases
patellofemoral joint syndrome see syndrome, runner's-knee
peroneal cuboid syndrome loss of rearfoot eversion due to long-standing peroneal tendon dysfunction/tendinitis; characterized by plantar pain from cuboid to first metatarsal
polycystic ovary syndrome see syndrome, Stein-Leventhal
Raynaud's syndrome concomitant Raynaud's disease (always affecting hands, and frequently feet) in patients with connective tissue disorders, characterized by generalized digital cyanosis, localized painful vasculitic lesions of dorsal forefoot (30% of cases) and apices of toes (20-25% of cases); subcutaneous calcinosis (20% of cases) may masquerade as a seed corn
Reiter's syndrome urethritis, iridocyclitis, arthritis, plantar enthesiopathy and heel spur formation, often triggered by earlier gastrointestinal Escherichia coli infection or exposure to a sexually transmitted disease (e.g. Chlamydia trachomatis); more common in human leukocyte antigen (HLA) B27 tissue-type males; see keratoderma blenorrhagicum
restless-leg syndrome overwhelming need to move the lower limbs constantly; characteristic of chronic renal failure; thought to be triggered by accumulation of metabolites and uraemia
Reye's syndrome cerebral oedema and death (in 50% of cases, usually children), provoked by aspirin therapy; aspirin is proscribed for children less than 16 years old
Riley-Day syndrome; familial dysautonomia autosomal-dominant complete indifference to pain; also characterized by orthostatic hypotension, hyperhidrosis and hyporeflexic/absent deep tendon reflexes, pes cavus and trophic plantar ulceration
Roussy-Levy syndrome; hereditary areflexic dystasia; Charcot-Marie-Tooth (CMT) disease type II essential tremor, sensory ataxia, poor coordination and judgement of movement, kyphoscoliosis and distal muscle atrophy (especially peronei); autosomal-dominant inherited disease similar to CMT disease type 1, but developing in early childhood
runner's-knee syndrome mild lateral subluxation of patella in patellar groove; due to an increase in Q angle (i.e. >15°), often in association with excessive foot pronation, tibial varum, internal tibial torsion, weakened quadriceps group, malposition of vastus medialis, hard running surfaces or faulty sports shoes, leading to uneven pressure on anterolateral surface of femoral condyle and local pain; often affects female runners; treated by prescription orthoses to reduce torque, torsion and knee joint stress
scalded-skin syndrome scaled/peeling appearance of skin overlying areas of infection, or associated with adverse drug reactions
'second-class travel' syndrome pulmonary thromboembolism due to prolonged periods of inactivity, e.g. passengers (who have been static for > 4 hours during long-haul intercontinental air flights) develop deep-vein thrombosis; the clot detaches, passing through venous circulation and heart, to block the pulmonary artery; characterized by sudden collapse and death; passengers on long-haul flights are advised to undertake leg muscle exercises regularly throughout the duration of the flight, wear 'antithrombotic' elasticated hosiery and consider medication with aspirin in the weeks before long-haul flight
sinus tarsi syndrome sensation of unsteadiness when walking on gravel/uneven ground and ongoing pain in lateral tarsal area just distal to and level with lateral malleolus, subsequent to inversion sprain/excess rearfoot pronation (e.g. as in rearfoot rheumatoid arthritis); local symptoms are exacerbated by heel inversion/eversion; treated by non-steroidal anti-inflammatory drugs, local immobilization, orthoses or steroid injection
SjÖgren's syndrome; sicca syndrome; keratoconjunctivitis sicca oral mucous membranes dryness, loss of lacrimal secretion, facial telangiectasias (i.e. butterfly rash), bilateral parathyroiditis (in younger women), strongly associated with rheumatoid arthritis and Raynaud's phenomenon
Stein-Leventhal syndrome; polycystic ovary syndrome multiple ovarian cyst formation, with associated menstrual abnormalities, infertility, enlarged ovaries, insulin resistance, obesity, acne, evidence of masculinization (e.g. hirsuitism) and increased tendency to type 2 diabetes mellitus; responds to treatment with oral contraceptive pill and/or metformin
Stevens-Johnson syndrome widespread bullous erythema multiforme of skin/mucous membranes; due to hypersensitivity/drug reaction
talar compression syndrome posterior ankle pain when foot is maximally plantarflexed at ankle joint; due to compression of posterior tubercle of talus on posterior margin of distal end of tibia; note: similar condition occurs with os trigonum, which impinges on posteroinferior margin of tibia (see Table 9)
tarsal tunnel syndrome; TTS pain, paraesthesia and numbness in sole of foot; due to tibial nerve compression within tarsal tunnel; associated with excess foot pronation or rearfoot rheumatoid arthritis; symptoms reproduced by tapping the skin overlying distal medial malleolar area (Tinel's sign positive); conservative treatment includes valgus filler pads, cobra pads and medial heel wedges, or control of excessive rearfoot pronation with moulded cushioned orthoses worn with bespoke shoes, together with non-steroidal anti-inflammatory drugs and/or disease-modifying antirheumatic drugs; surgical treatment includes decompression procedures to free posterior tibial nerve and excise local fibrous structures (see tarsal tunnel)
distal tarsal tunnel syndrome isolated entrapment of medial/lateral plantar nerves; medial plantar nerve is compressed between navicular tuberosity and belly of abductor hallucis longus, causing 'jogger's foot'; first branch of lateral plantar nerve (Baxter's nerve) may be entrapped as it courses laterally between bellies of abductor hallucis and quadratus plantae (flexor accessories) muscles (see Table 10)
proximal tarsal tunnel syndrome entrapment of posterior tibial nerve/its branches deep to flexor retinaculum; due to excessive subtalar joint pronation (with narrowing of tarsal tunnel, e.g. in rheumatoid foot) due to entrapment within attachments of flexor retinaculum, compression by an enlarged abductor hallucis muscle belly, enlarged navicular tuberosity, accessory navicular, presence of os tibialis externum, ischaemic compromise of posterior tibial nerve, or varicosities within tarsal tunnel
trisomy 21 syndrome see syndrome, Down's
Turner's syndrome sex-chromosome (XO) abnormality affecting 1:2500 females, with characteristic morphology (web neck, short stature), infantilism and amenorrhoea, coarctation of aorta and peripheral oedema; feet are oedematous, short and broad, show excess subtalar joint pronation and hyperextended halluces; nails tend to involution, and affected subjects are prone to ingrowing nails
Werner's syndrome autosomal-recessive condition characterized by scleroderma-like skin, cataracts, progeria (premature senility), hypogonadism and diabetes mellitus
Wernicke's syndrome; Wernicke-Korsakoff syndrome; Wernicke's encephalopathy brainstem ischaemia causing nystagmus and other ocular effects, tremors and ataxia, mental confusion, hypothermia and hypotension; more common in chronic alcoholics
Wolff-Parkinson-White syndrome congenital atrioventricular interconnection causing tachycardia and characteristic electrocardiogram pattern
yellow-nail syndrome see nail, yellow
Major criteria |
Current/historic Brighton score of 4/9 |
Arthralgia for >3/12 in four or more joints |
Minor criteria |
Current/historic Brighton score of 1, 2 or 3/9 (0, 1, 2, 3/9 if >50 years old) |
Arthralgia for minimum of 3 months in 1-3 joints, or back pain for minimum of 3 months, or spondylosis/spondylolysis/spondylolisthesis |
Dislocation/subluxation of > one joint, or one episode of simultaneous dislocation/subluxation of more than one joint |
Three or more lesions of soft-tissue rheumatism (e.g. spondylitis, tenosynovitis, bursitis) |
Marfanoid habitus (i.e. tall, slim physique, span:height ratio >1.3, upper:lower segment ratio <0.89, arachnodactyly [+Steinberg/wrist signs]) |
Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring |
Eye signs: drooping eyelids, myopia, antimongoloid slant |
Varicose veins or hernia or uterine/rectal prolapse |
Note: BFJHS is diagnosed in the presence of two major criteria, or one major and two minor criteria, or four minor criteria (adapted from Grahame R, Bird HA, Child A, Dolan AL, Fowler-Edwards A, Ferrell W, Gurley-Green S, Keer R, Mansi E, Murray K, Smith E. The British Society Special Interest Group on Heritable Disorders of Connective Tissue Criteria for the Benign Joint Hypermobility Syndrome. "The Revised (Brighton 1998) Criteria for the Diagnosis of the BJHS". Journal of Rheumatology 2000; 27:1777-1779). |
Phase | Features |
Acute phase (duration: 2-3 months) Reversible | Severe burning pain, warmth, swelling and joint stiffness within a limb: not confined to a dermatome or myotome Bone demineralization Symptoms (exacerbated by limb dependence, contact or stress) persist for 2-3 months |
Chronic phase (duration: several months) Reversible | Pain continues The limb becomes cool, firm and cyanotic Radiographs show diffuse osteoporosis Digits develop flexure contractures Persists for several months |
Atrophic phase Irreversible | Pain diminishes or becomes intractable Skin and subcutaneous tissues become atrophic Flexion contractures in foot become fixed Osteoporosis becomes advanced; bone has a 'ground-glass' appearance |
Major criteria |
Current/historic Brighton score of 4/9 |
Arthralgia for >3/12 in four or more joints |
Minor criteria |
Current/historic Brighton score of 1, 2 or 3/9 (0, 1, 2, 3/9 if >50 years old) |
Arthralgia for minimum of 3 months in 1-3 joints, or back pain for minimum of 3 months, or spondylosis/spondylolysis/spondylolisthesis |
Dislocation/subluxation of > one joint, or one episode of simultaneous dislocation/subluxation of more than one joint |
Three or more lesions of soft-tissue rheumatism (e.g. spondylitis, tenosynovitis, bursitis) |
Marfanoid habitus (i.e. tall, slim physique, span:height ratio >1.3, upper:lower segment ratio <0.89, arachnodactyly [+Steinberg/wrist signs]) |
Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring |
Eye signs: drooping eyelids, myopia, antimongoloid slant |
Varicose veins or hernia or uterine/rectal prolapse |
Note: BFJHS is diagnosed in the presence of two major criteria, or one major and two minor criteria, or four minor criteria (adapted from Grahame R, Bird HA, Child A, Dolan AL, Fowler-Edwards A, Ferrell W, Gurley-Green S, Keer R, Mansi E, Murray K, Smith E. The British Society Special Interest Group on Heritable Disorders of Connective Tissue Criteria for the Benign Joint Hypermobility Syndrome. "The Revised (Brighton 1998) Criteria for the Diagnosis of the BJHS". Journal of Rheumatology 2000; 27:1777-1779). |
Muscle group | Action (hold for 5-10 seconds; repeat ×5, three times a day) |
Hip abductor | Stand erect, legs straight, feet together; stretch trunk (on frontal plane) towards the unaffected leg |
Iliotibial band | Lie on a bench on the unaffected side, with the unaffected hip and knee slightly flexed, in order to maintain balance; flex the affected hip and straighten the affected knee so that the affected leg hangs off the bench; allow the iliotibial band of the affected leg to be stretched by gravitational pull Lie on a bench on the affected side with the affected leg in line with the body and the hip and knee locked; flex the unaffected (upper) leg; place the hands on the bench immediately under the shoulder and push the trunk upwards as far as possible to apply stretch to the lateral area of the affected leg |
Upper iliotibial band | Stand erect; with affected leg behind normal leg; stretch trunk (on frontal plane) towards unaffected side |
Lower iliotibial band | Stand erect as above, with the knee of the affected leg slightly flexed and hips rotated (on transverse plane) towards affected leg; stretch trunk (on frontal plane) towards the unaffected side |
Iliotibial band and hamstrings | Stand erect, with the affected leg behind the normal leg so that the knee of the affected leg rests on the posterior aspect of the non-affected knee; rotate the trunk (on transverse plane) away from the affected leg and attempt to touch the heel of the affected leg |
Visit | Action | |
1 | Examination Including Nobel's and Ober's tests, and excluding other causes of knee joint pain Gait analysis - walking and running | Check for presence of tibial varum, tibial torsion, uncompensated rearfoot varus and limb length discrepancy (include shoe wear pattern) Instigate the iliotibial band stretching regime (see Table 11), with a quadriceps- and adductor-strengthening programme Ice massage to painful area at lateral aspect of knee Advise reduction in athletic activity |
2 | Commence physical therapies, e.g. cortisone iontorphoresis or ultrasound and ice massage | Stabilizing orthoses and/or foot and ankle taping, ± heel lift Continue stretching programme ± massage Non-steroidal anti-inflammatory (10-day course of 400 mg ibuprofen qds) Stop all athletic activity if pain does not resolve |
3 | Magnetic resonance imaging/computed tomographic scan to knee joint area | Refer to orthopaedics |
Most cases will resolve with one treatment; more severe cases will require a second visit and some will require orthopaedic referral. |
Muscle group | Action (hold for 5-10 seconds; repeat ×5, three times a day) |
Hip abductor | Stand erect, legs straight, feet together; stretch trunk (on frontal plane) towards the unaffected leg |
Iliotibial band | Lie on a bench on the unaffected side, with the unaffected hip and knee slightly flexed, in order to maintain balance; flex the affected hip and straighten the affected knee so that the affected leg hangs off the bench; allow the iliotibial band of the affected leg to be stretched by gravitational pull Lie on a bench on the affected side with the affected leg in line with the body and the hip and knee locked; flex the unaffected (upper) leg; place the hands on the bench immediately under the shoulder and push the trunk upwards as far as possible to apply stretch to the lateral area of the affected leg |
Upper iliotibial band | Stand erect; with affected leg behind normal leg; stretch trunk (on frontal plane) towards unaffected side |
Lower iliotibial band | Stand erect as above, with the knee of the affected leg slightly flexed and hips rotated (on transverse plane) towards affected leg; stretch trunk (on frontal plane) towards the unaffected side |
Iliotibial band and hamstrings | Stand erect, with the affected leg behind the normal leg so that the knee of the affected leg rests on the posterior aspect of the non-affected knee; rotate the trunk (on transverse plane) away from the affected leg and attempt to touch the heel of the affected leg |
Grade | Characteristic |
1 | Pain on palpation of the anteromedial (or posteromedial) area of tibial crest No pain during activity or exercise |
2 | Pain after activity or exercise No pain during activity or exercise |
3 | Pain during activity or exercise Pain after activity or exercise |
4 | Pain and discomfort during normal walking Continual pain during activity or exercise |
Presentation | Treatment |
Phase 1: acute phase | Cessation of exercise activity until all pain resolves RICE(P) |
Phase 2: rehabilitation phase | Deep compartment muscle exercise to strengthen the deep fascial-bone interface and reduce tension on the deep fascial insertion, in order to decrease pain and swelling and prevent fascial scarring |
Phase 3: functional phase | Use of antipronatory/functional orthoses, strapping or taping in order to strengthen the fascial-bone interphase and prevent further excessive tension on the tibia |
Phase 4: return to activity | Phased and gradual return to normal levels of activity |
Neuroma/lesion | Involved nerve | Location |
Proximal tarsal tunnel syndrome | Branches of the posterior tibial nerve | Medial ankle area |
Distal tarsal tunnel syndrome Jogger's foot | Medial plantar nerve | Between navicular tuberosity and belly of abductor hallucis |
Distal tarsal tunnel syndrome Baxter's neuritis | Lateral plantar nerve | Between bellies of abductor hallucis, quadratus plantae and abductor digiti quinti minimi |
Joplin's neuroma | Medial plantar nerve proper | Medial area of first metatarsal head |
Houser's neuroma | First plantar intermetatarsal nerve | Between 1 and 2 metatarsals |
Heuter's neuroma | Second plantar intermetatarsal nerve | Between 2 and 3 metatarsals |
Morton's neuroma | Third plantar intermetatarsal nerve | Between 3 and 4 metatarsals |
Islen's neuroma | Fourth plantar intermetatarsal nerve | Between 4 and 5 metatarsals |
Accessory bone in the foot | Location |
Os tibiale externum (accessory navicular) | Within tibialis posterior tendon, adjacent to proximal part of navicular tuberosity |
Os trigonum | Posterior margin of talus |
Os peroneum | Within peroneus longus tendon, adjacent to inferior lateral border of cuboid/calcaneocuboid joint |
Os vesalianum | Adjacent to fifth metatarsal base |
Os intermetatarseum | Between bases of first and second metatarsals |
Os interphalangeus | Within insertion of flexor hallucis longus tendon, adjacent to plantar area of hallux interphalangeal joint |
Neuroma/lesion | Involved nerve | Location |
Proximal tarsal tunnel syndrome | Branches of the posterior tibial nerve | Medial ankle area |
Distal tarsal tunnel syndrome Jogger's foot | Medial plantar nerve | Between navicular tuberosity and belly of abductor hallucis |
Distal tarsal tunnel syndrome Baxter's neuritis | Lateral plantar nerve | Between bellies of abductor hallucis, quadratus plantae and abductor digiti quinti minimi |
Joplin's neuroma | Medial plantar nerve proper | Medial area of first metatarsal head |
Houser's neuroma | First plantar intermetatarsal nerve | Between 1 and 2 metatarsals |
Heuter's neuroma | Second plantar intermetatarsal nerve | Between 2 and 3 metatarsals |
Morton's neuroma | Third plantar intermetatarsal nerve | Between 3 and 4 metatarsals |
Islen's neuroma | Fourth plantar intermetatarsal nerve | Between 4 and 5 metatarsals |