polyarteritis nodosa (redirected from Kussmaul-Maier disease)

polyarteritis /poly·ar·ter·i·tis/ (-ahr″ter-i´tis)

1. multiple inflammatory and destructive arterial lesions.

2. polyarteritis nodosa.

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polyarteritis nodo´sa  (PAN) classically, a form of systemic necrotizing vasculitis involving small to medium-sized arteries with signs and symptoms resulting from infarction and scarring of the affected organ system.

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polyarteritis no·do·sa (n-ds)

n.

Systemic inflammation and necrosis occurring in medium-sized or small arteries. Also called Kussmaul's disease, periarteritis nodosa.

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polyarteritis nodosa,

a severe and poorly understood collagen vascular disease in which widespread inflammation and necrosis of small and medium-sized arteries and ischemia of the tissues they serve occur. Any organ or organ system may be affected. The disease attacks men and women between 20 and 50 years of age. Its cause is unknown, although immunologic factors are suspected. Polyarteritis nodosa may be acute and rapidly fatal or chronic and wasting. It is characterized by fever, abdominal pain, weight loss, neuropathy, and, if the kidneys are affected, hypertension, edema, and uremia. Some symptoms may mimic those of GI or cardiac disorders. Diagnosis is based on the clinical signs, results of laboratory tests, and findings of biopsy of sites affected by the disease. Mortality rate in polyarteritis nodosa is high, especially if there is kidney involvement. Aggressive treatment includes massive doses of corticosteroids. Immunosuppressive drugs have been used experimentally with some success. Physical therapy helps the patient to maintain muscle tone and prevents or slows the development of disability.

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polyarteritis

inflammatory and degenerative lesions of the arterial walls in a number of isolated locations.

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polyarteritis nodosa

See periarteritis nodosa.

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scleritis 

Inflammation of the sclera, which in its severe necrotizing or in the posterior type may cause sight-threatening complications such as keratitis, uveitis, angle-closure glaucoma or optic neuropathy. It affects females more commonly than males in the fourth to sixth decades of life. Like episcleritis it has a tendency to recur. It is characterized by pain, which can be severe, redness, tearing and some patients may develop nodules (nodular scleritis). It is often associated with a systemic disease (e.g. rheumatoid arthritis, Wegener's granulomatosis, polyarteritis nodosa, lupus erythematosus, ankylosing spondylitis, syphilis, herpes zoster). It can involve part of the sclera, e.g. anterior scleritis (which is the most common, and it is classified as diffuse non-necrotizing or nodular non-necrotizing) or posterior scleritis. Treatment includes topical and systemic steroids and immunosuppressive drugs for very severe cases. See acute stromal keratitis; Brown's superior oblique tendon sheath syndrome.
necrotizing scleritis The most severe form of scleritis, much less common than the other types. About half the patients have one of the following diseases: rheumatoid arthritis, Wegener's granulomatosis, polyarteritis nodosa, systemic lupus erythematosus, or herpes zoster. It is characterized by pain, and white, avascular areas next to damaged areas through which one can see the brown colour of the underlying uveal tissue, and to congested areas of the sclera. In most cases visual acuity is decreased. The necrosis gradually spreads around the globe. Treatment typically consists of topical steroids, immunosuppressive agents and occasionally surgery to repair scleral or corneal perforation. See keratolysis; scleromalacia.
scleritis necroticans See scleromalacia.
posterior scleritis Inflammation of the sclera involving the posterior segment of the eye. The condition is often associated with a systemic disease (e.g. rheumatoid arthritis). It is characterized by pain and reduced visual acuity. The severity of the visual impairment depends on the involved tissue and its location. Signs include eyelid oedema, proptosis, limitation of ocular movements and, if anterior scleritis is present, redness. The ocular fundus may present disc swelling, choroidal folds, macular oedema and serous retinal detachment. Treatment consists mainly of systemic steroids and immunosuppressive agents. See choroidal folds.

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polyarteritis nodosa

Periarteritis nodosa Internal medicine A connective tissue disease, characterized by necrotizing vasculitis of small to medium-sized arteries, affecting multiple organ systems–GI tract, kidneys, liver, muscle, skin; in > 50%, vasculitis affects peripheral nerves as mononeuritis multiplex or symmetric sensorimotor polyneuropathy Clinical Fever, malaise, myalgia Lab ↑ WBCs, ↑ ESR, 30-50% have HBsAg or HBsAb Management Immunosuppression–eg, prednisone, cyclophosphamide yields ± 90% 5-yr survival