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PURPOSE: We assessed the outcome of 31 patients with severe neuropsychiatric (NP) systemic lupus erythematosus (NPSLE) treated with intravenous cyclophosphamide (IV-CYC), and identified clinical predictors of response to therapy. METHODS: The authors performed a retrospective chart review and classified patients by NP manifestation and response to therapy as measured by serial anatomic imaging and neurodiagnostic studies coupled with clinical assessment of improvement. RESULTS: Neuropsychiatric manifestations occurred with the following frequencies: organic brain syndromes (OBS) 55%, stroke syndromes 35%, peripheral or mononeuropathy 32%, seizures 29%, psychiatric symptoms 26%, transverse myelitis 16%, cranial neuropathies 13%, other 16%. Most patients had multiple NP manifestations, with a median of two. Ninety percent of patients had failed therapy with corticosteroids with or without cytotoxic drugs prior to treatment with IV-CYC. Eight patients received synchronous plasmapheresis along with IV-CYC. After treatment with IV-CYC, NP deficits substantially improved in 61% (group I), stabilized in 29% (group S), and progressively deteriorated in 10% (group P). Patients in group I had significantly fewer NP manifestations than combined group S+P, two versus four, and a lower frequency of OBS, 37% versus 83%. CONCLUSION: Intravenous cyclophosphamide appears to be an effective treatment for some patients with severe NPSLE refractory to other forms of therapy. Higher number of NP manifestations and presence of OBS may predict poor outcome and identify a group of patients for whom early aggressive therapy may be indicated.

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The case is reported of a patient with central nervous system systemic lupus erythematosus (SLE) with features of progressive multifocal leucoencephalopathy (PML) seen clinically and by magnetic resonance imaging. A brain biopsy sample showed microinfarcts. The use of magnetic resonance imaging and IgG synthesis rates in evaluating central nervous system lupus, the co-occurrence of SLE and PML, and the differentiation of these entities by magnetic resonance imaging and by histology are considered.

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PURPOSE: To review the various rheumatologic manifestations of human immunodeficiency virus (HIV) infection and to discuss their potential pathogenic mechanisms. DATA IDENTIFICATION: A literature search using MEDLINE (1981 to 88) and Index Medicus (1981 to 88) and review of references from all identified articles. STUDY SELECTION: All studies and case reports addressing arthritis, myopathies, vasculitis, the sicca syndrome, systemic lupus erythematosus, and autoimmune phenomena in HIV-infected patients are cited. RESULTS OF DATA SYNTHESIS: The Reiter syndrome and other reactive arthritides are the commonest arthritides seen in HIV-infected patients. Psoriatic arthritis and septic arthritis with opportunistic agents, as well as an articular pain that is severe, acute, and intermittent, may also occur with HIV infection. An arthritis that may be specific for the acquired immunodeficiency syndrome (AIDS) has been described. Myopathies, especially of a polymyositic type, vasculitis, and the sicca syndrome are also part of the spectrum of rheumatologic presentation of HIV infection. Several autoimmune phenomena, such as antinuclear and anticardiolipin antibodies, as well as lupus anticoagulant, have been reported in HIV-infected patients. CONCLUSIONS: The Reiter syndrome, reactive arthritis, polymyositis, and the sicca syndrome may herald the onset of clinically evident HIV infection. These diseases and others may also occur in patients with full-blown AIDS. Furthermore, HIV infection may mimic systemic lupus erythematosus.

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A case of cerebral vasculitis in a previously healthy 22-year-old man with a history of cocaine abuse is described. Cerebral angiograms showed evidence of vasculitis. A search for possible causes other than cocaine produced no results. We now include cocaine with methamphetamines, heroin, and ephedrine as illicit drugs that can cause cerebral vasculitis.

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Streptococcus bovis bacteremia has been associated with several gastrointestinal disorders, most notably carcinoma of the colon. This report describes a 57-year-old woman with short bowel syndrome in whom S. bovis bacteremia and an infection of an indwelling parenteral nutrition catheter developed. A barium enema revealed diverticula and a foreshortened small intestine. This case implicates the short bowel syndrome in the pathogenesis of S. bovis bacteremia and supports empiric antibiotic coverage for both skin flora and enteric pathogens in patients with Hickman catheter sepsis and known gastrointestinal pathologic conditions.

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From 1982 to 1983 we received reports of a neurologic illness characterized by a symmetric descending paralysis in six drug abusers from widely separated geographic areas. Botulism was confirmed in two patients; type B botulinal toxin was found and Clostridium botulinum was isolated from a small abscess in one, and type A botulinal toxin was found in the serum of the other. The clinical illness in the remaining four patients, although not laboratory confirmed, was also compatible with botulism. None of the patients had histories suggestive of foodborne botulism, and wound botulism was suspected as the cause of illness. There are several reports of tetanus associated with parenteral drug abuse; wound botulism is another toxin-mediated clostridial infection that may occur as a complication of chronic drug abuse.

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The literature relating to prevalence, characteristics, differential diagnosis, histologic features, pathogenesis, complications, and prognosis of rheumatoid nodules is tabulated and reviewed. The broad spectrum of conditions associated with rheumatoid nodules is discussed. A new classification of these clinical entities, reflecting disease characteristics and prognosis, is proposed. This classification is based on age, association or lack thereof with rheumatoid arthritis or rheumatic features, the presence or absence of rheumatoid factors, and the location of the rheumatoid nodules. Four cases of rheumatoid nodules in patients without rheumatoid factor are presented. Three patients had classic or definite rheumatoid arthritis, and one had palindromic rheumatism. In follow-ups lasting one to 15 years, significant permanent joint deformities, bony erosions, or extra-articular manifestations of rheumatoid arthritis have not developed in any of the patients. All have experienced significant periods of remission.

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