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A6725 - Not so “Cryptogenic”: A Case of Organizing Pneumonia (OP) Associated with Common Variable Immunodeficiency (CVID)
Author Block: G. Manek1, L. Delasos1, C. Mosebach2; 1Internal Medicine, University of Connecticut School of Medicine, Farmington, CT, United States, 2Internal Medicine, University of Connecticut School of Medicine, Manchester, CT, United States.
Introduction: Immunodeficiency syndromes are linked with recurrent and chronic infections, including of the respiratory tract with one of the rare manifestations being organizing pneumonia. A thorough literature search revealed only seven reported cases of organizing pneumonia associated with Immunodeficiency syndromes. We present a case of organizing pneumonia associated with Common Variable Immunodeficiency. Case report: A 58 year old female with past medical history of diabetes mellitus presented with 2 day history of fever, chills and worsening dyspnea. She had multiple admissions in the past for pneumonia which recurred despite appropriate antibiotic therapy and extensive work up including CT imaging and lung biopsy established the diagnosis of organizing pneumonia (OP). Initially the patient’s organizing pneumonia was deemed cryptogenic as she was not on any medications associated with OP, had no history of toxic exposure, rheumatologic work up was negative for any auto immune process, malignancy work up for lung and hematologic malignancies were negative. She was initially treated with steroids which did not seem to improve her condition. During subsequent follow up, her immunoglobulin levels were checked which revealed the normal IgM levels, low IgG and IgA levels and patient was diagnosed with Common Variable Immunodeficiency (CVID). She was started on a course of intravenous immunoglobulin (IVIG) with marked clinical and radiological improvement. This admission, patient had vital signs significant for fever of 101, tachycardia, and tachypnea. Her physical exam was significant for diffuse crackles with superimposed wheezes bilaterally and laboratory work up revealed leukocytosis. Chest X ray demonstrated increased reticular markings associated with ground glass opacities and CT revealed bilateral ground glass opacities superimposed on coarse perihilar interstitial markings. Sputum culture, blood culture, Influenza PCR, Streptococcus PCR was unremarkable. Immunoglobulin levels were rechecked and found to be low. This was thought to be a flare up of her organizing pneumonia and she was treated with broad spectrum antibiotics, pulse dose steroids, IVIG with resolution of patient’s symptoms and follow up revealed resolution of chest X ray abnormalities. Discussion: Many cases of organizing pneumonia are eventually regarded as idiopathic. Organizing pneumonias are associated with connective tissue disorders, a variety of drugs, malignancies and rarely with immunodeficiency syndromes. This case illustrates the importance of exhaustive work up and considering a possible immunodeficiency in patients presenting with organizing pneumonia especially steroid resistant cases. Recognition and intervention with IVIG has been shown to improve symptoms and radiologic abnormalities.