Giant cell arteritis. A case with unusual neurologic manifestations and a normal sedimentation rate.

Giant cell (temporal) arteritis is a serious inflammatory condition that can lead to blindness, stroke, or other adverse sequelae if not properly treated. An elevated erythrocyte sedimentation rate has traditionally been emphasized as a criterion for making this diagnosis. Delays in diagnosis and unnecessary testing may occur when a patient presents with a normal erythrocyte sedimentation rate and a clinical history consistent with this condition. We describe a patient with giant cell arteritis who presented with a normal erythrocyte sedimentation rate and who subsequently developed devastating central nervous system complications.

Vasculitis with hepatitis B antigenemia: long-term observation in nine patients.

The development of generalized necrotizing vasculitis in association with hepatitis B antigenemia is the first example in man of a chronic rheumatic disease presumably caused by a viral infection. This report reviews the experience in nine biopsy-proven cases of hepatitis B-associated necrotizing vasculitis followed for up to six years. The natural history of the disease is emphasized and the manifestations of patients with vasculitis who carry hepatitis B antigen are compared with those of vasculitis patients who are antigen negative.

Pericardial disease in rheumatoid arthritis.

Six patients with rheumatoid constrictive pericarditis, five seen in a two and one half year period, are described. All patients were male, all had rheumatoid factor, and all had active arthritis. Diagnosis was suspected from careful physical examination and confirmed in five patients by cardiac catheterization. Pericardiectomy was successful in all five patients on whom it was performed. Rheumatoid constrictive pericarditis should be suspected in any patient with rheumatoid arthritis and unexplained signs of right heart failure.

Central nervous system disease in systemic lupus erythematosus. Therapy and prognosis.

The effect of corticosteroid therapy in 28 patients with 52 episodes of neuropyciatric disease in systemic lupus erythematosus (SLE) was elevated. Categories of organic central nervous system disease were seizures (eight patients), organic brain syndromes (nine patients), aseptic meningitis (four patients) and a variety of focal neurologic findings (seven patinets). Fourteen pateints had 15 episodes of functional psychosis without other evidence of neurologic disease. Although there was a general correlation between clinical and serologic evidnce of active SLE and the development of organic neurolgic disease, there was no evidence that therapy with very large doses of corticosteroids was beneficial. Of the deaths in this series, two were due to probable active SLE involving the central nervous system wheras five were attributable to complications of therapy. The long-term morbidity, likewise, was high in the patients who recieved large doses of corticosteroids. In all, 12 patients had major complications of corticosteroid therapy. Functional psychosis was usually preciptated by corticsoteroid therapy and respond to a reduction in steroid dosage and administration of psychotropic drugs.

Acute rheumatic fever.

A funny thing has happened on our way to the elimination of rheumatic fever from the United States. It is quite clear that, at least in some areas, rheumatic fever has made a dramatic resurgence. Although all the factors accounting for this are not clearly understood, there are several obvious conclusions that must be drawn: 1. Rheumatic fever is no longer the province of the poor and overcrowded. 2. Clinical manifestations, as embodied in the Jones criteria, may have changed somewhat over the years. 3. New insights into the genetics and immunology of rheumatic fever may lead to a vaccine and/or effective therapy in the future. 4. Rheumatic fever will continue to occur unless and until we resume an aggressive approach to the treatment and prophylaxis of pharyngitis, probably utilizing benzathine penicillin as our primary drug.

Suppression of systemic lupus erythematosus by the human immunodeficiency virus.

We describe a patient with refractory systemic lupus erythematosus (SLE) who demonstrated complete resolution of all SLE symptoms. He was subsequently found to be infected with the human immunodeficiency virus (HIV-1) and had marked depletion of peripheral CD4 positive T lymphocytes. In addition, while his SLE remained completely inactive, the course of HIV was rapidly progressive, suggesting that retroviral replication may have been enhanced by the underlying state of T cell transactivation characteristic of SLE.

Fibrinogen catabolism in systemic lupus erythematosus.

Because there is mounting evidence that localized intravascular coagulation may contribute to tissue injury following a variety of immunologic events, including immune complex diseases, fibrinogen catabolism was studied in patients with systemic lupus erythematosus to determine factors correlating with accelerated coagulation. 125I-fibrinogen half-life in controls was 90.1+/- 11 hours and the mean SLE half-life was 60.5 +/- 12. SLE patients in complete clinical remission had normal half-lives, but patients with symptomatic clinical disease, including renal disease, had significantly reduced fibrinogen survival. Accelerated fibrinogen consumption also correlated with positive tests for anti-DNA antibodies, but not with hypocomplementemia. These observations support the hypothesis that the coagulation system is activated in patients with immune complex diseases. Further studies are required to define the role, if any, that coagulation may play in causing tissue injury.

Fatal polymyositis in D-penicillamine-treated rheumatoid arthritis.

Thirteen reports of patients who developed polymyositis or dermatomyositis during treatment with D-penicillamine are reviewed and a fourteenth case is described. Twelve of the fourteen patients recovered after D-penicillamine was withdrawn; two patients died from cardiac involvement. Proximal muscle weakness was present in 13 patients and dermatomyositis in 4 patients. Dysphagia was the presenting symptom in 6 patients. Although D-penicillamine is useful in the management of rheumatoid arthritis, this drug should be used with caution and patients monitored closely for evidence of polymyositis or dermatomyositis.

Fibrinogen turnover in progressive systemic sclerosis.

Large amounts of fibrin are seen in the intima of the renal arterioles in progressive systemic sclerosis (scleroderma). The half-life or half disappearance time of plasma fibrinogen in 15 patients with scleroderma was studied using 125I fibrinogen to find whether there is an increased turnover of plasma fibrinogen paralleling this morphologic abnormality. Patients had a more rapid fibrinogen turnover than normal controls (60.7 versus 90.6 hours); the subgroup of patients with "progressive" scleroderma had a more rapid fibrinogen half-life than those with "stable" scleroderma (56.5 versus 73.2 hours). The mean fibrinogen half-life of 8 patients given intravenous heparin increased to within one standard deviation of normal, a finding that suggested that the fibrinogen molecule in these patients was capable of normal survival. There was a considerable variation of normal survival. There was a considerable variation of fibrinogen half-lives in individual scleroderma patients over time (not seen in the normal controls) which may be the result of intermittently increased fibrinogen consumption.

Normal distribution of acetylation phenotypes in systemic lupus erythematosus.

Previous reports have indicated that idiopathic systemic lupus erythematosus (SLE), like drug-induced lupus, is more frequent in "slow" hepatic acetylators. Using dapsone acetylation rate to determine phenotypes, we found that of the 18 SLE patients studied, 9 were fast acetylators, 8 were slow, and 1 was indeterminate. This result (53% fast) is similar to acetylator phenotypes in our normal controls (50% fast) and in the population at large (52%).

Rheumatoid arthritis and pure red cell aplasia.

Three patients with severe, deforming, and long-standing rheumatoid arthritis developed pure red cell aplasia that did not remit after withdrawal of medications, ran a chronic course, and in two patients remitted only after cytotoxic immunosuppressive treatment. An IgG inhibitor of autologous erythroid colony-forming and burst-forming unit growth in vitro was found in the serum of one patient. This specific erythropoietic inhibitor persisted in lower titer in the patient's serum even after an azathioprine-induced remission of pure red cell aplasia, indicating the possible need for maintenance immunosuppressive therapy. Chronic pure red cell aplasia may be another extra-articular manifestation of rheumatoid arthritis and should be considered when severe anemia develops in the absence of blood loss or hemolysis.

An account of the usefulness of a pilot clinical ethics program at a community hospital.

This article describes the development and implementation of a six-month pilot clinical ethics program at Saint Thomas Hospital (Nashville, Tenn). To assess the impact of this program, baseline data were gathered from a self-selected sample of critical and special care unit nurses and physicians about the "most troublesome" ethical dilemmas in their practices. Nurses and physicians reported facing similar dilemmas in practice. Nurses believed that chaplains and peers were most "beneficial" in resolving their "most troublesome" cases; physicians did not deem one particular individual or service to be of any greater benefit than any other in dilemma resolution. Nurses and physicians indicated that in many cases patients and families did not appear involved in the process. In a posttest survey following the pilot program, nurses rated the beneficial role of chaplains somewhat lower and agreed that the clinical ethics service was beneficial. As with the pretest sample, the posttest nurses evaluated the role of the attending physician as "detrimental" to resolving their ethical conflicts. In the posttest, physicians ranked the role of the clinical ethicist as comparable to that of chaplains and social workers.