Colchicine therapy in scleroderma. Preliminary results.
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Biomedical subjects
Publications and source records attributed to R A Frayha.
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In five males an erythema nodosum-arthropathy complex preceded the classical oro-genito-ocular triad of Behçet's disease by an average of 27 months. This symptom complex involving the legs initially suggested "idiopathic" erythema nodosum syndrome or acute sarcoidosis. As the diagnosis of Behçet's disease is clinical, these protean manifestations shoud be recognized as a possible "forme fruste" of the disease.
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Three patients with hereditary xanthinuria are presented and the pertinent literature is reviewed. In two siblings the disease has been asymptomatic; in the third urolithiasis has developed. Xanthine stone formation is the clinical hallmark of the disease. Hereditary xanthinuria seems to be relatively prevalent in Lebanon.
Two patients, a mother and daughter, each with the CRST syndrome, developed Sjögren's syndrome. Both patients had mitochondrial antibodies, smooth muscle antibodies, and a raised serum IgM without clinical evidence of liver disease. This family constitutes the first record of the familial coexistence of the CRST syndrome with Sjögren's syndrome, and the second evidence of vertical inheritance of scleroderma. It is suggested that patients with the CRST syndrome be studied for Sjögren's syndrome and for autoimmune liver disease.
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A young female came to the clinic with polymyositis and a biological false-positive serological test for syphilis (BFP reaction). Polymyositis, like other connective-tissue diseases, should be considered in the study of BFP reactors.
Two brothers with ochronosis are reported and the radiological features of ochronotic vertebral disease are reviewed. A striking feature was the presence of multiple vacuum discs. This "vacuum phenomenon" constitutes one of the earliest radiologic changes in the spine and presumably represents areas of severe degeneration within the intervertebral discs. Other features included a dark pigmentation of the urine after standing or following the addition of sodium hydroxide, and calcification within the cartilages of the ears and intervertebral discs. There was no abnormality of the peripheral joints and pigmentary deposits in the ears, sclerae, or skin were not observed.
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The unusual occurrence of polyarteritis nodosa presenting as non-giant cell temporal arteritis and a clinical picture suggestive of acute cholecystitis is reported.
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