systemic sclerosis


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Related to systemic sclerosis: progressive systemic sclerosis, CREST syndrome

sclerosis

 [sklĕ-ro´sis]
an induration or hardening, especially of a part from inflammation, or in disease of the interstitial substance. The term is used chiefly for such a hardening of the nervous system due to hyperplasia of the connective tissue or for hardening of the blood vessels. Called also induration. adj., adj sclerot´ic.
amyotrophic lateral sclerosis see amyotrophic lateral sclerosis.
arteriolar sclerosis arteriolosclerosis.
disseminated sclerosis multiple sclerosis.
familial centrolobar sclerosis a progressive familial form of leukoencephalopathy marked by nystagmus, ataxia, tremor, parkinsonian facies, dysarthria, and mental deterioration.
focal glomerular sclerosis focal segmental glomerulosclerosis.
glomerular sclerosis glomerulosclerosis.
hippocampal sclerosis loss of neurons in the region of the hippocampus, with gliosis; sometimes seen in epilepsy.
lateral sclerosis a form seated in the lateral columns of the spinal cord. It may be primary, with spastic paraplegia, rigidity of the limbs, and increase of the tendon reflexes but no sensory disturbances, or secondary to myelitis, with paraplegia and sensory disturbance.
medial calcific sclerosis (Mönckeberg's sclerosis) Mönckeberg's arteriosclerosis.
multiple sclerosis see multiple sclerosis.
systemic sclerosis systemic scleroderma.
tuberous sclerosis a congenital heredofamilial disease, transmitted as an autosomal dominant trait, characterized principally by the presence of hamartomas of the brain (tubers), retina (phakomas), and viscera, mental retardation, seizures, and adenoma sebaceum, and often associated with other skin lesions.

sys·tem·ic scle·ro·sis

1. a systemic disease characterized by formation of hyalinized and thickened collagenous fibrous tissue, with thickening of the skin and adhesion to underlying tissues (especially of the hands and face), dysphagia due to loss of peristalsis and submucosal fibrosis of the esophagus, dyspnea due to pulmonary fibrosis, myocardial fibrosis, and renal vascular changes resembling those of malignant hypertension; Raynaud phenomenon, atrophy of the soft tissues, and osteoporosis of the distal phalanges (acrosclerosis), sometimes with gangrene at the ends of the digits, are common findings. The term progressive systemic sclerosis is commonly used and is appropriate for cases with initially widespread skin involvement including the trunk. However, when skin involvement is limited to the distal extremities and face, there is often prolonged delay in appearance of visceral manifestations.
See also: CREST syndrome.
2. Synonym(s): scleroderma

systemic sclerosis

a form of scleroderma characterized by formation of thickened collagenous fibrous tissue, thickening of the skin, and adhesion of the skin to underlying tissues. The disease, which may be preceded by Raynaud's phenomenon, progresses to involve the tissues of the heart, lungs, muscles, genitourinary tract, and kidneys. See also scleroderma.

systemic sclerosis

Scleroderma Rheumatology An idiopathic connective tissue disease with clinical and pathologic features of GVHD, characterized by subcutaneous and visceral fibrosis, anticentromere and antitopoisomerase antibodies and prominent microvascular changes with endothelial cell damage and proliferation of subendothelial connective tissue; ♀:♂ ratio, 3-8:1-after child-bearing yrs Peak Age 45 to 55. See Connective tissue disease, Graft-versus-host disease.

systemic sclerosis

A generalized CONNECTIVE TISSUE disorder featuring FIBROSIS and degeneration of the skin and the internal organs. There is severe RAYNAUD'S PHENOMENON, swelling of the fingers, pigmentation, shiny atrophy and tightening of the skin so that the face becomes mask-like and there is difficulty in opening the mouth. There is no effective treatment.

Systemic sclerosis

A rare disorder that causes thickening and scarring of multiple organ systems.
Mentioned in: Scleroderma

systemic sclerosis

autoimmune connective tissue disease characterized by vasculitis and progressive sclerosis of soft tissues; causes vasculitic lesions of skin, joints, gut and kidney, calcinosis, malabsorption, arthritis, scleroderma and reduced kidney function
References in periodicals archive ?
Systemic sclerosis (SSc) is another disease belonging to the group of systemic connective tissue diseases.
Unlike systemic sclerosis, NSF tends to spare the skin of the face, and there is no association with autoantibodies or inflammatory cells.
Diffusing PuImonary Capacity Measured During Effort: A PossibIe EarIy Marker of Pulmonary Involvement In Systemic Sclerosis.
Bosentan reduces the number of new digital ulcers in patients with systemic sclerosis.
Creatine kinase, erythrocyte sedimentation rate, and C-reactive protein may be elevated due to myositis, vasculitis, malignancy, or an overlap of systemic sclerosis with another autoimmune disease.
Smit, "Skin autofluorescence, as marker of accumulation of advanced glycation endproducts and of cumulative metabolic stress, is not increased in patients with systemic sclerosis," International Journal of Rheumatology, vol.
This patient was included in systemic sclerosis patients group.
4 Thyroid dysfunction is commonly reported in systemic sclerosis but it has been rarely associated with morphea.
A few older papers suggested a possible link between gastric carcinoma and systemic sclerosis, on an atrophic gastritis and Helicobacter pylori (H.
Katrina, 32, ended up battling for her life after being diagnosed with a rare auto-immune disease, systemic sclerosis - a form of scleroderma that was slowly killing her.