Not Your Typical Case of Lupus Pneumonitis

Abstract

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary manifestations of Systemic Lupus Erythematosus (SLE) are varied and can affect any component of the respiratory tree. They include acute lupus pneumonitis (ALP), organizing pneumonia, interstitial lung disease and diffuse alveolar hemorrhage. We describe a unique case of lupus pneumonitis presenting as dense bilateral consolidation. CASE PRESENTATION: A 23-year-old African American female presented with rash, fatigue, arthralgias and dry cough. She was febrile, hypotensive, tachycardic and tachypnic. Physical exam was significant for oral ulcers, coarse rhonchi in bilateral lung fields, scaly hyperpigmented plaques on her face and erythematous macules on the lower extremities. She decompensated rapidly requiring intubation and was admitted for further evaluation. Initial laboratory analysis were significant for pancytopenia, mild transaminitis, a protein gap and proteinuria. Chest imaging showed dense consolidation in the lower lobes (Image 1). The patient underwent a bronchoscopy, which noted clear airways without pus or blood; sequential alveolar aliquots were not consistent with alveolar hemorrhage. An extensive infectious workup was negative; however, autoimmune workup was positive (Table 1). A clinical diagnosis of SLE was made. The patient failed to improve on antibiotics; low dose steroids were added with no change. Acute lupus pneumonitis was considered given the negative infectious workup and the persistent lower lobe opacities. She was started on pulse dose steroids with rapid radiographic resolution and clinical improvement, facilitating extubation. DISCUSSION: Acute lupus pneumonitis (ALP) is a life-threatening syndrome occurring in 1—4% of patients with SLE.1 The diagnosis can be elusive as the clinical presentation is non-specific. While chest x-ray can show patchy acinar infiltrates, usually with a lower lung zone predominance, dense bilateral consolidation has not been previously well described.2 Additionally, our patient did not have evidence of diffuse alveolar hemorrhage on bronchoscopy nor findings consistent with organizing pneumonia, which can both present with patchy alveolar opacities. She only improved after initiation of pulse dose steroids for ALP. CONCLUSIONS: Clinicians should consider ALP in patients presenting with dense consolidations, negative infectious workup and clinical suspicion of SLE. The overall prognosis for ALP is poor with 50-90% mortality despite treatment; early diagnosis is of utmost importance to provide the best chance of recovery.3 Reference #1: Torre, Olga, and Sergio Harari. “Pleural and Pulmonary Involvement in Systemic Lupus Erythematosus.” Pulmonary Involvement in Systemic Disease (2011). Reference #2: Kim, Jeung Sook et al. “Thoracic Involvement of Systemic Lupus Erythematosus: Clinical, Pathologic, and Radiologic Findings.” Journal of Computer Assisted Tomography 24.1 (2000): 9-18. Reference #3: Murin, Susan et al. “Pulmonary Manifestations of Systemic Lupus Erythematosus.” Clinics in Chest Medicine 19.4 (1998): 641-65. DISCLOSURE: The following authors have nothing to disclose: Bhavika Kaul, Simon Yau, Venkata Bandi No Product/Research Disclosure Information