Abstract Introduction Common variable immunodeficiency (CVID) is the most common of the primary immunodeficiency disorders. It’s caused by impaired B-cell differentiation and low immunoglobulin count. It is usually present in ages 20-40 and rarely ever in elderly patients. Pulmonary manifestations include recurrent sinopulmonary infections and bronchiectasis. Less commonly, the lung may be affected by lymphoid disorders and sarcoid-like granulomas and rarely cryptogenic organizing pneumonia (COP). Case Presentation We present a case of a 70 year old lady with a past medical history of childhood recurrent sinusitis and bronchitis, asthma, breast cancer post lumpectomy and chemotherapy in 2018, recurrent admissions for culture negative, antibiotic resistant pneumonias recurring after steroid tapering who presented with chronic worsening dyspnea admitted initially for possible pneumonia. She was hemodynamically stable with a normal WBC count and negative autoimmune workup. CT scan of chest showed multilobar consolidations with no nodularity or cavitation (Figure 1A). Sputum cultures and respiratory panel were negative. Despite broad-spectrum antibiotics, her symptoms continued to worsen and oxygen requirements progressively increased. Ultimately, flexible bronchoscopy was decided for her with bronchoalveolar lavage being negative for infection, and transbronchial biopsy showed COP (Figure 1B), She was started on steroids with significant clinical, radiographic improvement. During her hospitalization, serum immunoglobulin levels revealed low IgG (375 mg/dl), Ig A (10 mg/dl), normal IgM and IgE levels. She had poor response to strep pneumonia panel and poor response to PPSV23 vaccination. Given those findings along with her history of recurrent sinopulmonary infections, a diagnosis of CVID was made. She started on IVIG and very slow prednisone taper. She was referred to genetics for further immunodeficiency panel testing. Discussion COP is a rare presentation in CVID and both are rare to present in elderly patients. The main differential for COP is LIP (lymphoid interstitial pneumonia) which presents similarly and is associated with SLE, RA, Sjögren’s, CVID, and infections especially HIV. Also, LIP presents with cystic airspaces and nodules on CT scan with the absence of granulation plugs and polyps on biopsy as was the case with our patient. The mechanism of OP in CVID is unclear but likely involves epithelial injury leading to fibroblast activation and fibrin deposition with dysregulated VEGF/MMP-mediated angiogenesis linked to immune dysregulation and recurrent infections. This abstract is funded by: None
Za’Nouneh et al. (Fri,) studied this question.