Medicine
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This paper reports the experiences of our group with 68 patients with progressive systemic sclerosis (PSS) admitted to hospitals of the University of Pittsburgh Health Center between 1955 and 1981 with scleroderma renal crisis (SRC). The onset of SRC was characterized by four features, namely, onset or aggravation, usually abrupt, of arterial hypertension; appearance of Grade III or IV retinopathy; elevations of peripheral renin activity to at least twice the upper limit of normal; and rapid deterioration of renal function within a period of less than one month. Over 90% of our patients in whom these criteria could be determined had at least three of them present with the onset of SRC. ⋯ Progress in the last few years seems to have been achieved primarily by the advent of pharmacologic agents that specifically block the effect of angiotensin II by inhibiting the angiotensin I converting enzyme. When diagnosis is prompt and the condition is treated as an emergency with these compounds, we and others have found that normal renal function can be restored in a number of patients. The result is a considerably brighter outlook for patients with this previously rapidly fatal complication of progressive systemic sclerosis.
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Forty-nine patients with disseminated gonococcal infection (DGI) hospitalized at Boston City and University Hospitals over a 7-year period were studied. Patients with clinical manifestations of DGI and with cervical, urethral, rectal, pharyngeal, synovial or blood cultures positive for Neisseria gonorrhoeae were separated into two groups based on the presence or absence of suppurative arthritis. There were 19 cases of suppurative arthritis (Group II) and 30 cases with only tenosynovitis, skin lesions, or both (Group I). ⋯ Eighteen of 24 Group I strains (75%) versus 9 of 19 Group II strains (47%) resisted killing by all normal human sera tested (p less than .05). Likewise, convalescent sera from Group II patients were able to kill their infecting strains more often than did sera from Group I patients (70% vs 17%) (p less than 0.01). Thus, variations in the clinical expression of disease in patients with DGI may be explained, in part, by differences in certain phenotypic and immunologic features of infecting strains.
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Comparative Study
Serological findings in patients with "ANA-negative" systemic lupus erythematosus.
Serological studies were performed on sera from 66 patients with the clinical picture of systemic lupus erythematosus (SLE). These sera failed to give a positive antinuclear antibody test when tested on cryostat sections of mouse liver and thus these patients' sera appear to be ANA negative. Precipitating antibodies to the cytoplasmic antigen Ro were found in 41 cases and of the remaining 25 sera, 18 were found to have antibodies to single stranded DNA detectable by radioimmunoassay. ⋯ The clinical picture of these patients was dominated by a severe photosensitive dermatitis but more than half of the patients had widespread multisystem disease. As a group these patients had a low frequency of nephritis and neuropsychiatric disease. Detection of these antibodies relates these patients serologically to other SLE patients and suggests that they are best perceived as part of the clinical spectrum of SLE.