Incidental Finding to Diagnostic Dilemma

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Lymphocytic Interstitial Pneumonitis (LIP) is a common manifestation of lung disease associated with Sjogren’s disease. We present a case fraught with diagnostic challenges. CASE PRESENTATION: A 59 year old man with history of Sjogren’s syndrome and asthma was referred to pulmonology after incidental X-ray finding of right lower lobe infiltrate. Review of systems revealed dry eyes, dry cough and dyspnea on exertion. He had a 25 pack-year smoking history. Medications were notable for saline eye drops and Albuterol inhaler. Physical examination revealed temperature 37 degrees Celsius; blood pressure 145/77; pulse 61; oxygen saturation 96% on room air. He was obese, with no lymphadenopathy and chest clear to auscultation. Initial Lab work was unremarkable. Serology was positive for antinuclear antibodies (ANA), anti Anti-Sjögren’s- syndrome-related antigen A and antigen B (SSa/SSb) antibodies. Pulmonary function tests revealed a mildly restrictive pattern. Initial CT scan showed right upper lobe infiltrate which worsened on 3 month follow up CT. Bronchoscopy with bronchoalveolar lavage (BAL) showed normal cell count and cytology, cultures grew beta streptococcus group B. After 2 weeks of antibiotics he developed night sweats and dyspnea on mild exertion. A third CT scan demonstrated dense right lower lobe consolidation, bilateral ground-glass opacities and enlarged sub-pleural nodules. Repeat BAL grew Cryptococcus. He received 7 weeks of fluconazole without improvement. Needle biopsy for sub-pleural nodule revealed multiple dense lymphoplasmacytic infiltrates. Ultimately, wedge biopsy confirmed dense lymphoplasmacytic infiltrate, lymphoid follicles, intra-alveolar macrophages and histiocytes. B-cell polyclonality was confirmed by PCR. He was diagnosed with focal lymphoid hyperplasia and lymphocytic interstitial pneumonia. Treatment with corticosteroids yielded transient resolution. Dramatic resolution was achieved with Rituximab (with azathioprine for maintenance). DISCUSSION: The pathogenesis of LIP is thought to be abnormal proliferation of indigenous cell lines and lung parenchymal infiltration with lymphoid cells. It may be a focal or diffuse, benign (monoclonal) or potentially malignant (polyclonal). It is most commonly seen in women in their 50s and is associated with autoimmune disease. Frequently, LIP presents with cough, chest pain and hemoptysis. There may be a restrictive ventilatory defect on PFTs. Imaging is variable and a lung biopsy required for diagnosis. CONCLUSIONS: Timely recognition of LIP prevents misdiagnosis. Treatment usually is with high dose corticosteroids. Alternative to steroid use may be immunosuppressant agents such as rituximab and azathioprine, however literature to support use of such agents is limited to case reports. Reference #1: Lymphocytic Interstitial Pneumonia, Tanmay S. Panchabhai MD, Carol Farver MD, Kristin B. Highland MD, MSCR Clinics in Chest Medicine, 2016-09-01 DISCLOSURE: The following authors have nothing to disclose: Reema Qureshi, Saira Imran No Product/Research Disclosure Information