Lupus Vasculitis

The spectrum of Systemic Lupus Erythematosus (SLE) includes latent lupus, discoid lupus, drug-induced lupus, neonatal lupus, lupus profundus, neuropsychiatric lupus, lupus vasculitis, pulmonary lupus, etc. The disease course is characterized by unpredictable exacerbations, drug-induced remissions and spontaneous remissions. SLE is characterized by a wide range of variable individual clinical manifestations which are controllable at early stages. 

Type III IgG and IgM Vasculitis, serum sickness, lupus Penicillins, sulfonamides, radiocontrast agents,... 

Pyelonephritis, interstitial nephritis Glomerulonephritis, renal infard, lupus nephritis, vasculitis... 

For some autoimmune diseases, little is known about environmental factors involved in the initiation or progression of the disease. For other diseases, however, considerable research has been conducted on one or more types of exposures. Most epidemiologic studies of environmental influences have focused on multiple sclerosis, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, and small vessel vasculitis, but experimental studies using murine models of these diseases is limited (Table 25.1). 

Examples include vasculitis, lupus-like reactions, glomerulonephritis, hemolytic anemia. 

Dapsone is approved for the treatment of an autoimmune blistering skin disease, dermatitis herpetiformis. This intensely pruritic eruption is characterized histologically by a dense dermal infiltration of neutrophils and subepidermal blisters. Other skin diseases in which dapsone is helpful are linear immunoglobulin A (IgA) dermatosis, subcorneal pustular dermatosis, leukocytoclastic vasculitis, and a variety of rarer eruptions in which neutrophils predominate, including some forms of cutaneous lupus erythematosus. 

Thalidomide is approved for use in the United States for the treatment of cutaneous manifestations of erythema nodosum leprosum, a potentially life-threatening systemic vasculitis that occurs in some patients with leprosy. Although not approved for other indications, thalidomide has also been shown to be very effective in the management of Behget s disease, HIV-related mucosal ulceration (aphthosis), and select cases of lupus erythematosus. 

The main clinical uses of immunosuppressive drugs are suppression of organ and tissue rejection after transplant surgery and the treatment of diseases with an autoimmune component. Thses include renal diseases, e.g. glomerulonephritis, some nephrotic syndromes, connective tissue diseases, such as systemic lupus erythematosus rheumatoid arthritis, and systemic vasculitis. 

Cyclophosphamide is active against rheumatoid arthritis when given orally at dosages of 2 mg/kg/d but not when given intravenously. It is used regularly to treat systemic lupus erythematosus, vasculitis, Wegener s granulomatosis, and other severe rheumatic diseases. 

MMF is effective for the treatment of renal disease due to systemic lupus erythematosus and may be useful in vasculitis and Wegener s granulomatosis. Although MMF is occasionally used at a dosage of 2 g/d to treat rheumatoid arthritis, there are no well-controlled data regarding its efficacy in this disease. 

Other rheumatic diseases in which the corticosteroids potent anti-inflammatory effects may be useful include vasculitis, systemic lupus erythematosus, Wegener s granulomatosis, psoriatic arthritis, giant cell arteritis, sarcoidosis, and gout. 

Adverse reactions to the thioamides occur in 3-12% of treated patients. Most reactions occur early, especially nausea and gastrointestinal distress. An altered sense of taste or smell may occur with methimazole. The most common adverse effect is a maculopapular pruritic rash (4-6%), at times accompanied by systemic signs such as fever. Rare adverse effects include an urticarial rash, vasculitis, a lupus-like reaction, lymphadenopathy, hypoprothrombinemia, exfoliative dermatitis, polyserositis, and acute arthralgia. Hepatitis (more common with propylthiouracil) and cholestatic jaundice (more common with methimazole) can be fatal, although asymptomatic elevations in transaminase levels also occur. 

Antithyroid drugs, especially propylthiouracil, can be associated with the development of antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis, often manifesting as renal disease. Atypical presentations, with pulmonary capillaritis (58) and lupus-like syndrome (59), have been described in individual cases. Furthermore, two cases of vasculitis have been associated with carbimazole, one presenting with eosinophilic granulomatous vasculitis localized to the stomach (60) and another with p-ANCA positive vasculitis causing simultaneous acute renal insufficiency and massive pulmonary hemorrhage (61). 

Several cases of collagen-like or lupus-like disease have been reported (joint pain, skin rash, and positive antinuclear antibodies) during treatment with either propylthiouracil or thiamazole (SEDA-8, 372) (SEDA-10, 368). Some cases of general vasculitis can be fatal, although high-dose glucocorticoid therapy can be helpful (90). 

Although the most common methotrexate dosing regimens for the treatment of rheumatoid arthritis are 15 or 17.5 mg weekly, there is an increased effect up to 30 or 35 mg weekly. The drug decreases the rate of appearance of new erosions. Evidence supports its use in juvenile chronic arthritis, and it has been used in psoriasis, psoriatic arthritis, polymyositis, dermatomyositis, Wegener s granulomatosis, giant cell arteritis, subacute lupus erythematosus, and vasculitis. 

One controlled, double-blind trial plus anecdotal evidence attest to the efficacy of chlorambucil in rheumatoid arthritis. Chlorambucil has also been used in Behef s disease, systemic lupus erythematosus, vasculitis, and other autoimmune disorders. 

Steinman, C. R., Circulating DNA in systemic lupus erythematosus Association with central nervous system involvement and systemic vasculitis. Am. J. Med. 67, 429-435 (1979). 

Hydralazine has been in use since the 1950s and is usually used in combination with other drugs such as diuretics and P-blockers. In a significant number of patients, and typically after 18 months, adverse effects started to appear. These included joint and muscle pain (arthralgia and myalgia), a rash on the face and inflamed blood vessels (vasculitis). The rash on the face made afflicted patients look wolf-like, which gave rise to the name for the syndrome. Lupus erythematosus (Lupus is Latin for wolf). This disease can be caused by other drugs, such as isoniazid very occasionally and procainamide more frequently. It may also have other, unknown, causes and has some similarities with rheumatoid arthritis. 

The thionamide drugs are all liable to cause minor and major adverse effects. Minor are rash, urticaria, arthralgia, fever, anorexia, nausea, abnormalities of taste and smell. Major are agranulocytosis, thrombocytopenia, acute hepatic necrosis, cholestatic hepatitis, lupus-like syndrome, vasculitis. 

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