Published online February 1, 2006
PEDIATRICS Vol. 117 No. 3 March 2006, pp. e590-e595 (doi:10.1542/peds.2005-1349)
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EXPERIENCE AND REASON

Sarcoidosis in Chronic Granulomatous Disease

Suk See De Ravin, MD, PhDa, Nora Naumann, MDa, Michael R. Robinson, MDb, Karyl S. Barron, MDa, David E. Kleiner, MD, PhDc, Jean Ulrick, RNa, Julia Friend, PA-Ca, Victoria L. Anderson, MSNa, Dirk Darnell, MSNa, Elizabeth M. Kang, MDa and Harry L. Malech, MDa

a National Institute of Allergy and Infectious Diseases
b National Eye Institute
c National Cancer Institute, National Institutes of Health, Bethesda, Maryland

In addition to increased susceptibility to infections in patients with chronic granulomatous disease (CGD), a higher incidence of sterile inflammatory disorders in these patients has been noted. However, sarcoidosis has not been reported previously in CGD. In this report, we describe two patients who have CGD and a disorder consistent with sarcoidosis on the basis of unequivocal clinical-radiographic presentations, their responses to treatment, and serum angiotensin-converting enzyme levels. Serum angiotensin-converting enzyme levels were measured in 26 other patients with CGD to establish an appropriate reference range. A possible relationship between CGD and sarcoidosis is discussed.

Key Words: chronic granulomatous disease • sarcoidosis • neurosarcoidosis • angiotensin-converting enzyme

Abbreviations: CGD, chronic granulomatous disease • ACE, angiotensin-converting enzyme • CT, computed tomography • ESR, erythrocyte sedimentation rate • SLE, systemic lupus erythematosus • TNF-{alpha}, tumor necrosis factor {alpha} • Th1, helper type 1 T cells

Accepted Aug 15, 2005.


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