Journal Article

Fulminant Mulch Pneumonitis: An Emergency Presentation of Chronic Granulomatous Disease

Sophia Siddiqui, Victoria L. Anderson, Diane M. Hilligoss, Mario Abinun, Taco W. Kuijpers, Henry Masur, Frank G. Witebsky, Yvonne R. Shea, John I. Gallin, Henry L. Malech and Steven M. Holland

in Clinical Infectious Diseases

Published on behalf of Infectious Diseases Society of America

Volume 45, issue 6, pages 673-681
Published in print September 2007 | ISSN: 1058-4838
Published online September 2007 | e-ISSN: 1537-6591 | DOI: http://dx.doi.org/10.1086/520985
Fulminant Mulch Pneumonitis: An Emergency Presentation of Chronic Granulomatous Disease

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Background. Chronic granulomatous disease (CGD) is associated with multiple and recurrent infections. In patients with CGD, invasive pulmonary infection with Aspergillus species remains the greatest cause of mortality and is typically insidious in onset. Acute fulminant presentations of fungal pneumonia are catastrophic.

Methods. Case records, radiograph findings, and microbiologic examination findings of patients with CGD who had acute presentations of dyspnea and diffuse pulmonary infiltrates caused by invasive fungal infection were reviewed and excerpted onto a standard format.

Results. From 1991 through 2004, 9 patients who either were known to have CGD or who received a subsequent diagnosis of CGD presented with fever and new onset dyspnea. Eight patients were hypoxic at presentation; bilateral pulmonary infiltrates were noted at presentation in 6 patients and developed within 2 days after initial symptoms in 2 patients. All patients received diagnoses of invasive filamentous fungi; 4 patients had specimens that also grew Streptomyces species on culture. All patients had been exposed to aerosolized mulch or organic material 1–10 days prior to the onset of symptoms. Cases did not occur in the winter. Five patients died. Two patients, 14 years of age and 23 years of age, who had no antecedent history of recognized immunodeficiency, were found to have p47phox-deficient CGD.

Conclusions. Acute fulminant invasive fungal pneumonia in the absence of exogenous immunosuppression is a medical emergency that is highly associated with CGD. Correct diagnosis has important implications for immediate therapy, genetic counseling, and subsequent prophylaxis.

Journal Article.  4542 words.  Illustrated.

Subjects: Infectious Diseases ; Immunology ; Public Health and Epidemiology ; Microbiology

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