Cryptococcosis in systemic lupus erythematosus: a series of six cases

Abstract

The aims of this study were to describe the clinical features of patients with systemic lupus erythematosus (SLE) who developed cryptococcal infection and ascertain their outcomes when treated with glucocorticoids and immunosuppressive agents in conjunction with long-term maintenance antifungal therapy. Six cases of cryptococcal infection in SLE were reviewed retrospectively. The mean age at the time of infection was 26.3 (11.7) years. Three patients had active SLE and all were receiving glucocorticoids [median prednisone dose of 40 (21.2-60.0) mg/day] at the time of infection diagnosis. Concomitant cytotoxic agents were used in five patients. Meningitis was the most common clinical manifestation (n = 5) and cryptococcemia was found in three cases. The patient, who developed pulmonary cryptococcosis, died from respiratory distress syndrome. All patients received induction anti-fungal therapy with amphotericin B and the five surviving patients switched to oral fluconazole indefinitely as maintenance therapy and none of them has had relapses of cryptococcal infection to last medical evaluation. As SLE patients have intrinsic abnormalities of cell-mediated immunity and receive immunosuppressive therapy, indefinite maintenance therapy with fluconazole is recommended in SLE patients with cryptococcosis.

Pulmonary Cryptococcosis

“…HIV infection is the major risk factor for cryptococcosis. Other populations at risk include solid-organ and hematological stem cell transplant recipients; patients with malignancies 26,27 ; those receiving immunomodulating and biologic agents, including patients treated for systemic lupus erythematosus (SLE), 28,29 sarcoidosis, 30 and rheumatoid arthritis 31 ; and individuals with CD4 lymphopaenia. 32,33 These risk factors have been described in both C. neoformans and C. gattii infections.…”

“…The impact of HIV on the immune response to cryptococcosis is also reviewed by Chang et al 54 Complement and mannose-binding lectin deficiency, impaired chemotaxis and phagocytosis of macrophages and polymorphonuclear cells, and abnormal T-cell-mediated cytotoxicity associated with SLE and glucocorticoid use contribute to the pathogenesis of cryptococcosis. 29…”

Cryptococcal meningitis in systemic lupus erythematosus patients: pooled analysis and systematic review

“…1 The most common risk factors for infection in SLE patients are the use of high-dose corticosteroids, antibiotics exposure, 2 high SLE activity and intrinsic disorders of cell-mediated immunity. 3 Moreover, as SLE is a systemic disease, ~20%–70% of SLE patients develop central nervous system (CNS) damage due to autoimmune-mediated attack, 4 making the CNS susceptible to infection through impairment of the blood–brain barrier.…”

Application of the DiversiLab system for tracing the source of the mixed infections caused by Cryptococcus neoformans var. grubii from a patient with systemic lupus erythematosus

“…Many studies have revealed the propagules of C. neoformans and C. gattii floating in the air can be inhaled by human beings . Some people are resistant to the infectious propagules, but others unfortunately develop cryptococcosis . Transmission of cryptococcosis from human to human was rarely found .…”