Rheumatoid vasculitis

Primary systematic vasculitis Rheumatoid disease Sjogren s syndrome Behcet s disease... 

Extra-articular involvement may include rheumatoid nodules, vasculitis, pleural effusions, pulmonary fibrosis, ocular manifestations, pericarditis, cardiac conduction abnormalities, bone marrow suppression, and lym-phadenopathy. 

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). 

Unlabeled Uses Treatment of rheumatoid vasculitis, heavy metal toxicity. 

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. 

Indomethacin was particularly popular for gout and ankylosing spondylitis. In addition, it has been used to accelerate closure of patent ductus arteriosus. Indomethacin has been tried in numerous small or uncontrolled trials for many other conditions, including Sweet s syndrome, juvenile rheumatoid arthritis, pleurisy, nephrotic syndrome, diabetes insipidus, urticarial vasculitis, postepisiotomy pain, and prophylaxis of heterotopic ossification in arthroplasty. 

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. 

Rheumatic arthritis, rheumatoid arthritis, cervical or lumbar spondylosis, osteoarthritis, sciatica, Raynaud s disease, vasculitis, pulmonary heart disease, rheumatic heart disease, coronary heart disease, migraine, hemiplegia after cerebrovascular accident, paralysis, facial paralysis, facial spasm, skin diseases that are influenced by changes in the weather and are characterized by itchy, weeping, red or dry skin lesions. 

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. 

R2. Rapoport, R. J., Kozin, F., Macket, S. E., and Jordon, R. E., Cutaneous vascular immunofluorescence in rheumatoid arthritis Correlation with circulating immune complexes and vasculitis. Am. J. Med. 68, 325-331 (1980). 

W9. Weisman, M., and Zvaiffler, N., Cryoimmunoglobulinemia in rheumatoid arthritis. Significance in serum of patients with rheumatoid vasculitis. /. Clin. Invest.56, 725-739 (1975). 

Rheumatoid disease is rarely complicated by a systemic vasculitis, which can involve the brain (Genta et al. 2006). Occasionally atlanto-axial dislocation causes symptomatic vertebral artery compression (Howell and Molyneux 1988). 

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. 

Rheumatoid vasculitis affecting the CNS is rare but may present as seizures, dementia, hemiparesis, cranial nerve palsy, blindness, hemispheric dysfunction, cerebellar ataxia or dysphasia (Vollertsen and Conn, 1990 Ando et al., 1995) Expression of... 

A comprehensive review discusses the therapeutic management of RA (Turesson and Matteson, 2004). Epidemiological studies link extra-articular rheumatoid arthritis manifestations with premature mortality and support aggressive anti rheumatoid therapies for those patients. Cyclophosphamide is favored in patients with systemic rheumatoid vasculitis and methotrexate in those cases with other manifestations of extra-articular rheumatoid arthritis (Turesson and Matteson, 2004). Cyclophosphamide and TNFa inhibitors such as infliximab have some positive success in treatment resistant vasculitis associated with RA (Unger et al., 2003). However, TNFa inhibitors have also been associated with the opposite effect, an induction of extra articular rheumatoid arthritis so their use should be used only in specific cases when close monitoring is in place. 

Unger L, Kayser M, Nusselsein HG (2003) Successful treatmerrt of severe rheumatoid vasculitis by infliximab. Ann Rheum Dis 62 587-588. 

Vollertserr RS, Corrrr DL (1990) Vasculitis associated with rheumatoid ar tlrritis. Rlreum Dis Clirr North Am 16 445 61... 

In addition to its use for rheumatoid arthritis, azathoiprine is employed for its steroid-sparing effect in many autoimmune diseases, as an immxmo-suppressant, e.g. after organ transplant, and to maintain remission in the treatment of vasculitis. Other aspects of azathiopurine are discussed elsewhere in the book (see Index). 

In extreme severity, high-dose prednisolone (20-40 mg/d) will very effectively suppress inflammation, e.g. with vasculitis or rheumatoid lung. 

Alemtuzumab (campath-lH) is a humanized monoclonal antibody specific for the CDw52 antigen, present on cell membranes of lymphocytes and monocytes. It has been used for treatment of patients with rheumatoid arthritis and vasculitis, is being investigated for the treatment of chronic lymphocytic leukemia, and has been used to deplete circulating lymphocytes in patients with multiple sclerosis (1). In 2001, alemtuzumab was approved in Europe for the treatment of chronic B cell lymphocytic leukemia that had been treated previously with alkylating agents and was refractory to fludarabine (2). It has also been used for induction of immunosuppression/tolerance in liver transplant recipients (3,4) and kidney/pancreas transplant recipients (5). 

In a placebo-controlled study in 48 patients with active rheumatoid arthritis, CD4 blockade produced clinical benefit (5). Adverse events were reported in 97% of the patients, compared with 73% of those given placebo. In both groups most of the events were mild to moderate. Serious adverse events were reported in five patients who received anti-CD4 syncope/vasovagal attacks (n = 3), back pain (n = 1), abdominal pain/rectal bleeding (n = 1). Skin rashes occurred in 62% of the patients who received the antibody. In five cases a skin biopsy was performed, and showed a cellular infiltration centered on the blood vessels, suggesting a drug-induced vasculitis. 

There have been several descriptions of new cutaneous or pulmonary nodulosis in patients with rheumatoid arthritis treated with etanercept (4,18) concomitant cutaneous vasculitis was also reported in two patients (18). Although this may have been due to the natural history of rheumatoid arthritis or a lack of response to treatment, the short time to the occurrence of cutaneous nodulosis after the start of therapy in some patients implicated the etanercept. 

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