TOPIC: Pulmonary Vascular Disease
TYPE: Medical Student/Resident Case Reports
INTRODUCTION: We present a case of a middle-aged female with antiphospholipid syndrome (APS) presenting as atypical pneumonia and alveolar hemorrhage later confirmed to have APS-induced vasculitis.
CASE PRESENTATION: 65 years female with APS with positive anti-cardiolipin beta2 glycoprotein antibodies presented to the Emergency Department (ED) with complaints of worsening dyspnea, chronic cough, intermittent fever over the last 2-3 weeks. She had oxygen saturation of 85%, fever of 101 F with rest vitals stable. Lung exam revealed diffuse bilateral crackles. Lab showed pancytopenia. Diffuse non-consolidated bilateral opacities was seen in chest imaging. Few hours later, she developed respiratory failure requiring intubation. She was started on pulse steroid and broad-spectrum antibiotics for atypical pneumonia. Bronchoscopy yielded evidence of hemorrhage. Infectious workup and other serology were negative. She improved clinically & underwent video-assisted thoracoscopic surgery for wedge biopsy of lung before discharge. Biopsy results were followed which showed necrotizing capillaritis with patchy intra-alveolar hemorrhage, hemosiderin-laden macrophages and fibrin.
DISCUSSION: APS is an autoimmune condition with persistently elevated antiphospholipid antibodies (lupus anticoagulant, anticardiolipin or anti-B2-glycoprotein-I) commonly manifests with systemic thrombosis. These antibodies may result in pulmonary microvascular injury thus rarely (2%) presents as diffuse alveolar hemorrhage (DAH) . In patients presenting with DAH, underlying etiology can be diagnosed through a detailed history and physical, pertinent serology, chest imaging, bronchoscopy with lavage, and biopsy in case of uncertain diagnosis. Out of 3 major pathological findings in DAH (pulmonary capillaritis, bland hemorrhage, or diffuse alveolar damage), pulmonary capillaritis is closely associated with autoimmune diseases and thus frequently identified in patients with APL (55%); but absence of capillaritis does not rule out APS . Lung biopsy in pulmonary capillaritis may show neutrophilic infiltration of the pulmonary capillary and hemorrhage within the alveoli. Capillaritis may be observed more often than microvascular thrombosis ( 60% vs 11% vs 29% combined) . All patients with capillaritis have strong positive titer of aPL . Diagnosis can be made with biopsy after correlating with clinical presentation, laboratory data along with immunofluorescence studies. Diagnosis is challenging in case of transbronchial biopsy because of small tissue samples . Routine lung biopsy is not required for diagnostic confirmation given the risk-benefit ratio as these patients are at a higher risk of thrombosis or bleeding due to anticoagulation [1, 2].
CONCLUSIONS: DAH may be an initial presentation of APS. Typical biopsy findings are sufficient to establish the diagnosis of small vessel vasculitis given the high pretest probabilities.
REFERENCE #1: Stoots SA, Lief L, Erkan D. Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome. Curr Rheumatol Rep. 2019;21(10):56. Published 2019 Sep 6. doi:10.1007/s11926-019-0852-7
REFERENCE #2: Cartin-Ceba R, Peikert T, Ashrani-- A, Keogh K, Wylam ME, Ytterberg S, Specks U. Primary antiphospholipid syndrome-associated diffuse alveolar hemorrhage. Arthritis Care Res (Hoboken). 2014 Feb;66(2):301-10. doi: 10.1002/acr.22109. PMID: 23983016.
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