title: Mucormycosis in systemic autoimmune diseases.
abstract: Mucormycosis is an emerging infection in systemic autoimmune diseases. All published cases of systemic autoimmune diseases complicated by mucormycosis were reviewed. The clinical features, diagnostic procedures and the main principles of treatment were analyzed. Twenty-four cases of mucormycosis have been reported in systemic auto-immune diseases, of which 83% in systemic lupus erythematosus, all occurring during immunosuppressants. In most cases, the infection was disseminated or rhinocerebral and it had mimicked a flare of the underlying connective tissue disease. A fatal outcome was reported in 58.3% of these patients. In conclusion, mucormycosis often mimics a flare of the underlying systemic disease and is associated with a high mortality rate. Systemic lupus erythematosus is by far the most common associated systemic autoimmune disease. A high degree of awareness is warranted to rapidly rule out infection, of which mucormycosis, in immunocompromised patients with systemic autoimmune disease before a disease flare is conclusively diagnosed.
authors.affiliation: Service de rhumatologie, CHU d'Angers, 49933 Angers cedex 9, France.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Royer
authors.affiliation: Inserm U1016, CNRS UMR 8104, institut Cochin, Paris, France; National Referral Center for Rare Systemic Autoimmune Diseases hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France. Electronic address: xavier.puechal@cch.aphp.fr.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000009:adverse effects* / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D009091:Mucormycosis / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology / Q000628:therapy*; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: Immunosuppressive drugs; Mucormycosis; Opportunistic infections; Systemic autoimmune diseases