Elsevier

Seminars in Arthritis and Rheumatism

Volume 33, Issue 2, September–October 2003, Pages 115-124
Seminars in Arthritis and Rheumatism

Mucormycosis in systemic lupus erythematosus

https://doi.org/10.1016/S0049-0172(03)00081-7Get rights and content

Abstract

Objective: To describe a case of mucormycosis in systemic lupus erythematosus (SLE) and to review other patients reported in the English literature.

Method: A Medline search for articles about mucormycosis in SLE published between 1970 and 2002 was performed by using the key words “lupus,“ “mucormycosis,” “zygomycosis,“ “Mucorales,” “Rhizopus,” and “Mucor.” Cases were pooled for analysis, and the mycology, diagnosis, treatment, and outcome of mucormycosis in SLE was reviewed.

Results: Eight cases of mucormycosis in SLE were identified (female:male = 7:1). The mean age at the time of infection was 31.8 ± 7.6 years and the mean duration of SLE was 6.3 ± 3.9 years. All except 1 patient had active lupus and all were receiving high-dose corticosteroids. Concomitant cytotoxic agents were used in 4 patients. Additional predisposing factors for opportunistic infection included hypocomplementemia, nephrotic syndrome, uremia, leukopenia, and diabetes mellitus. The disseminated form of mucormycosis was the most common presentation and the diagnosis often was made only at autopsy (63%). For cases with positive culture results, Rhizopus was the causative species. In 4 patients, manifestations of the fungal infection mimicked those of active SLE. The overall mortality of mucormycosis was very high (88%) and, in most cases, was probably a function of delayed diagnosis and treatment. The cutaneous form appeared to have the best prognosis with combined medical and surgical treatment.

Conclusions: Mucormycosis is a rare but usually fatal fungal infection in SLE. Judicious use of immunosuppressive agents, a high index of suspicion, early diagnosis, and combination treatment with amphotericin B and surgical debridement may improve the prognosis of this serious infection.

Section snippets

Methods

A patient with SLE who presented with headache and multiple cranial nerve palsies in the setting of a nephritic flare is reported. She was initially treated for active neuropsychiatric lupus with heavy immunosuppression. However, her condition deteriorated rapidly and a final diagnosis of extensive rhinocerebral mucormycosis was made at autopsy. Culture of a nasal swab specimen yielded Rhizopus species.

A Medline search for articles published between 1970 and 2002 regarding mucormycosis in SLE

Case report

A 36-year-old Chinese woman was admitted in June 2000 because of fever, arthritis, and generalized anasarca caused by nephrotic syndrome. SLE was diagnosed in 1990 when she presented with photosensitive facial rash, polyarthritis, and diffuse proliferative nephritis, for which high-dose prednisone and intravenous pulse cyclophosphamide were administered. Her nephritis remitted after cyclophosphamide treatment, and she remained stable throughout these years while maintained on low-dose

Mycology

Mucormycosis is a rare opportunistic infection caused by fungi of the order Mucorales (class Zygomycetes) (26). Most infections are caused by Mucor, Rhizopus, Absidia, and Rhizomucor species from the family Mucoraceae, and rarely by Cunninghamella from the family Cunninghamellaceae and other species. Apophysomyces elegans, a newly recognized genus and species classified in the family Mucoraceae, has been recently reported to cause mucormycosis 27, 28, 29. The fungi are ubiquitous in the

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  • Cited by (0)

    1

    Chi Chiu Mok, MD, MRCP: Consultant in Rheumatology, Department of Medicine, Tuen Mun Hospital, Hong Kong

    2

    Tak Lun Que, FRCPA, FRCPath: Senior Consultant, Department of Microbiology, Tuen Mun Hospital, Hong Kong

    3

    Edmund Yik Kong Tsui, MBBS, FRCR: Consultant, Department of Radiology, Tuen Mun Hospital, Hong Kong

    4

    Wing Yin Lam, MBBS, FRCPath, FIAC: Senior Consultant, Department of Pathology, Tuen Mun Hospital, Hong Kong, China.

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