SESSION TITLE: Miscellaneous Cases - Student/Resident
SESSION TYPE: Student/Resident Case Report Slide
PRESENTED ON: Tuesday, October 27, 2015 at 04:30 PM - 05:30 PM
INTRODUCTION: Bronchiectasis with recurrent infections, often with atypical organisms, should suggest an underlying immunodeficiency. Auto-antibodies directed against cytokines involved in host defense are a recently described cause of immunodeficiency. A subset of auto-antibodies has been associated with thymoma and thymic malignancies.
CASE PRESENTATION: A 71 year old man presented to pulmonary clinic for evaluation of a chronic cough. His cough was present for 20 years, with daily sputum production and recurrent episodes of hemoptysis. His past medical history was significant for a malignant thymoma resection in 2006 with adjuvant radiation and chemotherapy. Chest CT revealed bronchiectasis in the left upper lobe along his radiation field, which was managed with standard therapy and inhaled tobramycin for recurrent sputum cultures positive for pseudomonas. After an increase in hemoptysis, a BAL culture grew aspergillus fumigatus. He was also diagnosed with esophageal candidiasis. HIV test was negative. One year later, he again had increasing sputum production and grew norcardia asteroides in sputum cultures. Given his recurrent atypical infections, he underwent further testing which revealed undetectable levels of CD20+ cells, and a low percentage of CD4 cells. Immunoglobulin levels were normal making Good Syndrome unlikely. Bone marrow biopsy was unrevealing. A serum sample sent to the National Institutes of Health revealed antibodies to INF-alpha, INF-omega, and IL12p70 and IL22. The patient was diagnosed with immunodeficiency secondary to anti-cytokine autoantibodies related to thymic malignancy. He is managed with prophylactic antibiotics and standard therapy for bronchiectasis.
DISCUSSION: Our patient had bronchiectasis with recurrent atypical pulmonary infections. Routine evaluation including serum immunoglobulins, HIV, and CF testing were normal. Immunology evaluation eventually led to the diagnosis of anti-cytokine auto-antibody syndrome. Anti-cytokine syndromes range from pulmonary alveolar proteinosis (anti-GM-CSF) to pure red cell aplasia (anti-EPO), and more recently, acquired immunodeficiency from anti-IFNγ. Thymic disease is often associated with auto-immune conditions and now is recognized to be associated with anti-cytokine antibodies, leading to a clinical syndrome of recurrent opportunistic infections. Some patients have been successfully treated with anti-CD20 (rituximab) therapy, though our patient had undetectable CD20+ cells suggesting that rituximab would be of little clinical benefit.
CONCLUSIONS: High rates of auto-antibodies to cytokines are described in patients with thymic malignancies, leading to immunodeficiency and opportunistic infections. Awareness is important in particular when evaluating adult onset immunodeficiency.
Reference #1: Burbelo PD, Browne SK, Sampaio EP et al. Anti-cytokine autoantibodies are associated with opportunistic infection in patients with thymic neoplasia. Blood. 2010 Dec 2;116 (23):4848-58.
DISCLOSURE: The following authors have nothing to disclose: Anita Oh, Alex Kim, Taylor Doherty, Rebecca Sell
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