The spectrum of polymyositis and dermatomyositis.
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Biomedical subjects
Publications and source records attributed to A Bohan.
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A case granulomatous liver disease associated with the polymyalgia rheumatica-giant cell arteritis syndrome is presented. Treatment with corticosteroids resulted in prompt improvement in both the polymyalgia rheumatica-giant cell arteritis syndrome, as well as the tests of liver function. It is suggested that polymyalgia rheumatica and giant cell arteritis may involve the liver and should be included in the differential diagnosis of granulomatous liver disease.
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Upon careful examination, 23 of 24 patients with progressive systemic sclerosis (PSS) were found to have abnormalities of muscle. Nineteen patients presented a homogenous pattern of muscle abnormalities, which untreated was associated with a stable course ("simple myopathy"). Three patients demonstrated inflammatory muscle disease indistinguishable from polymyositis while a fourth patient developed marked weakness associated with a generalized neuropathic process. Muscle enzymes, electromyogram, and muscle biopsy permitted distinction among the different muscle disorders, a distinction that could have prognostic and therapeutic importance.
The diagnosis of systemic lupus erythematosus (SLE) often depens on positive tests for antinuclear antibody, LE cells, and antibody to DNA. In fact, the presence of such serological markers is virtually required for the diagnosis of active disease. This report describes 5 well-studied patients with SLE who initially presented with negative serology despite active multisystemic disease. When followed from 10 months to 7 yr, negative serologic markers, including the ANA, LE cell test, and anti-DNA were seen to change from negative to positive, analogous to the seronegative rheumatoid patient who may become seropositive. In a few cases of lupus, therefore, negative serologic tests do not always exclude SLE, and serial testing at periodic intervals may be indicated.