Cryptococcal meningitis in systemic lupus erythematosus.

Two cases of cryptococcal meningitis occurring in patients with systemic lupus erythematosus (SLE) are presented, and 24 additional cases from the literature are reviewed. The insidious onset of this infrequent complication is emphasized. The nonspecific neurological findings associated with this infection are often mistakenly diagnosed as a central nervous system manifestation of SLE. Earlier diagnosis and effective antifungal therapy have improved the prognosis of cryptococcal meningitis in SLE patients in recent years. Strategies for the treatment of patients with this complication are discussed.     

Trimethoprim-sulfamethoxasole induced meningitis in systemic lupus erythematosus.

Most reports of drug induced meningitis in systemic lupus erythematosus (SLE) have implicated ibuprofen. We describe a 46-year-old woman with SLE who developed aseptic meningitis abruptly after ingesting trimethoprim-sulfamethoxasole (TMP-SMX). This patient had received TMP-SMX twice before; each was associated with increasingly severe reactions, whose relationship with the use of TMP-SMX became apparent only in retrospect. A history of medication use should be sought in all patients with meningitis who have an underlying autoimmune disorder.     

Aseptic meningitis in systemic lupus erythematosus

Three patients with aseptic meningitis were subsequently diagnosed as having lupus erythematosus. One patient had a single meningitic episode, another had chronic meningitis, and the third two acute episodes 5 years apart. All 3 patients developed further neuropsychiatric manifestations of SLE, leading to death in 1. Aseptic meningitis appears to be an early manifestation of SLE and may herald more serious brain damage. No new cases of aseptic meningitis occurred in this series after initiation of therapy for SLE. In contrast, bacterial meningitis did occur as a late complication of the disease.     

Transverse myelopathy in systemic lupus erythematosus. Report of three cases and review of the literature.

Three cases of transverse myelopathy associated with systemic lupus erythematosus were reported, and 23 similar cases previously reported were reviewed. A diagnosis of systemic lupus erythematosus was made in only 60% before the onset of transverse myelopathy. The time of onset of myelopathy was randomly distributed during the disease. The most common presenting neurologic symptom was numbness, or weakness of the legs, or both. A unique association between the acute stage of transverse myelopathy and marked reduction of cerebrospinal fluid glucose concentration was observed. Thirteen patients died, 9 had permanent neurologic deficits, and only 4 recovered nearly normal function. Eight patients had ischemic necrosis of the spinal cord at postmortem examination, and vascular lesions were found in the spinal cord of 3 additional patients. The value of steroid treatment was uncertain. Patients who were started on steroid therapy within 24 hours of the onset of myelopathy may have benefited.     

Pulmonary hypertension in systemic lupus erythematosus: a report of three cases

Three women with systemic lupus erythematosus (SLE) who developed pulmonary hypertension as a terminal feature of their illness, are reported. One patient with quiescent SLE had marked sicca syndrome when pulmonary hypertension developed. Two of the 3 patients possessed the "lupus anticoagulant" and antibodies to cardiolipin. All 3 patients had positive rheumatoid factor in their sera.     

Immune complex deposition and coronary vasculitis in systemic lupus erythematosus. Report of two cases

Extramural coronary arteries were examined in two patients with systemic lupus erythematosus (SLE). Coronary vasculitis was found in both patients. One patient with clinically and serologically inactive SLE had died suddenly and was found to have a myocardial infarction secondary to the coronary vasculitis. Immunopathologic studies demonstrated immune reactants in the walls of inflamed and noninflamed arterial segments in a pattern consistent with immune complex aggregates. Immunologic injury secondary to immune complex deposition may be responsible for the development of coronary disease in patients with SLE. This has been demonstrated in experimental animals but not in humans. Although this is an uncommon complication of SLE, it represents a cause of sudden death and a potentially treatable lesion in this patient population. Its occurrence may be related to the deposition of immune aggregates in the walls of coronary vessels.     

Deep fungal infection in systemic lupus erythematosus - three cases reported, literature reviewed.

Three patients with systemic lupus erythematosus (SLE) and deep fungal infection are described. Two patients had disseminated cryptococcal infection and the third disseminated histoplasmosis. Allwere receiving corticosteroids at the time fungal infection developed. One patient with disseminated cryptococcosis improved after treatment with amphotericin B and 5-fluorocytosine. The other patient with disseminated cryptococcosis died before adequate therapy could be given. The patient with histoplasmosis responded satisfactorily to amphotericin B. A survey of the literature revealed 30 additional cases of deep fungal infection in patients with SLE, most of whom were on corticosteroid therapy. The majority of the patients had candidiasis (14 patients); 11 patients had severe cryptococcal infection. Other fungal infections reported were histoplasmosis, aspergillois, coccidioidomycosis, and maduromycosis caused by Allerscheria boydii. Twenty-seven patients died; in 22 death was related to the fungal infection. The fungal infection was not diagnosed until necropsy in at least 11 persons. Deep fungal infections should be considered whenever patients with SLE have fever of unknown origin, diffuse pulmonary infiltrates, or unexplained CNS symptoms.     

JC virus meningitis in a patient with systemic lupus erythematosus

The human neurotropic JC virus (JCV) is most commonly acquired during childhood, and, because no clinical illness has been associated with primary infection, is presumed to be asymptomatic. In the immunocompromised host, JCV is responsible for progressive multifocal leukoencephalopathy (PML). We describe a patient with longstanding systemic lupus erythematosus who presented with acute meningitis without encephalitis or PML. JCV was the only pathogen found in the cerebrospinal fluid suggesting a primary infection or symptomatic reactivation. Our observation demonstrates the expanding clinical importance of JCV in autoimmune diseases, and diagnostic tests for JCV should be included in the investigative work-up for meningitis or encephalitis in these patients.     

Cerebellar ataxia in systemic lupus erythematosus: three case reports.

Three patients presented with cerebellar ataxia among 350 cases of systemic lupus erythematosus (SLE) seen over the last 14 years. Cerebellar signs were unilateral in one and bilateral in the other two patients. Other neurological findings were present in all three patients. One initially presented with only cerebellar ataxia; other features of SLE appeared a few years later. Lupus anticoagulant test was positive in one patient. Corticosteroids given in the early stages appeared to benefit these patients by ameliorating cerebellar dysfunction.     

Use of high-dose intravenous immunoglobulin in systemic lupus erythematosus: report of three cases

Three patients with life-threatening manifestations of systemic lupus erythematosus (SLE), unresponsive to conventional high-dose corticosteroid and/or immunosuppressive therapy were treated with intravenous polyspecific IgG (IVIG). Following IVIG infusion, lupus encephalitis in the first patient quickly resolved and the impressive improvement of the clinical status was associated with a transient increase in C1q-binding activity. The daily infusion of IgG had to be suspended after three days in the second patient with encephalitis and nephritis, because the renal function rapidly deteriorated; subsequently, six plasma exchanges led to an almost complete recovery. Finally, leukocyte and platelet counts increased and remained within normal range following IgG therapy in the third patient having SLE-associated leuko- and thrombocytopenia. In all three patients a decrease in anti-DNA antibody levels and an increase in total complement hemolytic activity were detected after therapy.     

Treatment of severe and difficult cases of systemic lupus erythematosus with tacrolimus. A report of three cases

OBJECTIVES—An analysis of the efficacy of tacrolimus treatment in three patients with difficult and severe systemic lupus erythematosus (SLE) whose active disease had been previously poorly controlled by cyclosporine and cyclophosphamide.
METHODS—A review of patient notes.
RESULTS—Two patients are well controlled after six and nine months of treatment with tacrolimus 0.06 mg/kg/day and 0.18 mg/kg/day. Previous persistent vasculitis had resolved and other features of active disease were controlled. The third patient's vasculitis had not improved significantly after two months of treatment and tacrolimus 0.17 mg/kg/day was discontinued because of nephrotoxicity.
CONCLUSION—Tacrolimus may be a useful additional immunosuppressive agent in some patients whose SLE is not well controlled by conventional treatments.

     

Listeria monocytogenes meningitis in a young woman with systemic lupus erythematosus

Listeria meningitis, a rare but life-threatening infection in patients with systemic lupus erythematosus, often represents a diagnostic and therapeutic challenge because of its rarity and non-representative manifestations. L. monocytogenes is an intracellular pathogen capable of spreading directly from cell to cell without exposure to the extracellular humoral immune system. With the evolving trend of intense immunosuppressive therapy, patients with SLE usually have abnormal cell-mediated immunity and are susceptible to L. monocytogenes infections. The gastrointestinal tract is usually the portal of entry, and a transient gastroenteritis may precede the full-blown meningitides. Ampicillin and penicillin G are the drugs of choice. For patients who are allergic to penicillin, trimethoprim-sulfamethoxazole is an eligible alternative. Delay in diagnosis and inappropriate antibiotics are detrimental to the outcome. Herein, we report a young woman with systemic lupus erythematosus who developed listeria meningitis. Clinicians are advised to be aware of the clinical presentations of this disease.     

Ascites in systemic lupus erythematosus.

Peritoneal serositis is not a widely recognised aspect of systemic lupus erythematosus (SLE). Indeed, ascites in SLE is said to occur only when complicated by the nephrotic syndrome, congestive cardiac failure, or hepatic cirrhosis. We describe two patients who developed ascites that could be attributed to none of these complications.     

Fever in systemic lupus erythematosus.

The frequency, causes, clinical and laboratory features, and outcome of febrile episodes in 160 hospitalized patients with systemic lupus erythematosus were reviewed. Eighty-three febrile episodes were identified in 63 patients and were ascribed to active lupus erythematosus alone (60 per cent), infections (23 per cent) and miscellaneous causes (17 per cent). Bacteremia was present in nine of the 19 infectious episodes and resulted in a fatal outcome in a third of the patients. Leukocytosis, neutrophilia, shaking chills and normal levels of anti-DNA antibodies were associated with infection in febrile patients with lupus erythematosus.     

ANA-negative systemic lupus erythematosus.

The presence of antinuclear antibodies (ANA) in serum is generally considered a decisive diagnostic sign of systemic lupus erythematosus (SLE). Ten patients with clinical signs of disease but persistent negative tests for ANA are examined in this study. Hair fall, Raynaud's phenomenon and recurrent oral ulcers were common in the ANA-negative group. ANA-negative SLE seems to be a subgroup of SLE that has not previously been given adequate attention.