Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I |

Granulomatosis With Polyangiitis: Unmasked FREE TO VIEW

Geetanjali Johri, MD; Patrick Kohlitz, MD; Bisma Alam, MD
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SUNY Upstate Medical University, Syracuse, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1079A. doi:10.1016/j.chest.2016.08.1186
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a condition that is mimicked by other illnesses and given its low prevalence in the United States at approximately 3 out of 100,000 cases; it is often placed equally low on the differential list. It is important not to delay this diagnosis as it can lead to end-organ damage if left untreated.

CASE PRESENTATION: 52-year-old male with history of psoriatic arthritis and recent sinus infection presented with sweats, fatigue, weight loss, dyspnea, productive cough, and hemoptysis for one week. Patient had significant social history including 30-pack-years of smoking, recent travel to 40 states, and exposure to tuberculosis. Shortly prior to admission, he was tapered off Prednisone and Methotrexate for psoriatic arthritis due to hepatotoxicity, and was started on Adalimumab monotherapy. On presentation, patient was febrile, tachycardic, with leukocytosis, and acute kidney injury. He had multiple purpuric lesions and bronchial breath sounds in the right lung. Imaging revealed a hilar mass, cavitary lesions, calcified nodules, and post-obstructive consolidation. Patient was treated empirically for pneumonia and disseminated fungal infection. He underwent bronchoscopy with biopsy of hilar mass and washings. Serologies including cANCA and antiproteinase-3 were positive. As concern for infection persisted, immunosuppressive therapy could not be initiated for suspected vasculitis. Kidney function rapidly worsened and transient dialysis was required. Lung, kidney, and skin biopsy revealed necrotizing granulomas with negative stain for mycobacteria and fungi. GPA was confirmed given the constellation of clinical and serological findings. Anti-infective agents were discontinued. Patient was started on treatment and his pulmonary and renal manifestations stabilized with Prednisone and Cyclophosphamide.

DISCUSSION: Methotrexate with steroid therapy is proven to effectively control symptoms of GPA. A study done showed that 89% improved with this therapy and 74% achieved remission. It is thus likely that our patient was misdiagnosed with psoriatic arthritis based on musculoskeletal symptoms alone. He likely suffered from GPA with symptoms that surfaced at the discontinuation of steroids and Methotrexate.

CONCLUSIONS: Our patient presented with the telltale signs of GPA including fatigue, weight loss, sinus disease, acute renal failure, and purpuric skin lesions. He had several confounding factors (traveling history, tobacco use, recent immunosuppression, anchoring bias) that resulted in the delay of diagnosis. It is important to keep in mind that many diseases may mimic GPA and despite its low prevalence in the US, it should be recognized early for prompt treatment to prevent life-threatening complications.

Reference #1: Seo P, Stone JH. Antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004; 117:39.

Reference #2: Puéchal, X., et al. Long-Term Outcomes in the WEGENT Trial. Arthritis Rheum 2016, 68: 690-701.

DISCLOSURE: The following authors have nothing to disclose: Geetanjali Johri, Patrick Kohlitz, Bisma Alam

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