"Possible Primary Antiphospholipid Syndrome" with Concurrent Diffuse Alveolar Hemorrhaging and Libman-Sacks Endocarditis Mimicking Catastrophic Antiphospholipid Syndrome

Abstract

Although antiphospholipid antibody syndrome (APS) is an autoimmune condition that is primarily characterized by arterial or venous thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies (aPL), recent reviews have introduced non-thromboembolic manifestations. We describe the case of a 58-year-old woman with vegetation on the aortic valve, whose initial presentation of APS abruptly developed into diffuse pulmonary hemorrhage. Despite consecutive plasma exchange procedures and the administration of corticosteroids and high-dose intravenous immunoglobulin, multiple brain infarctions developed, and the patient died of pneumonia. Although anecdotal, this case might serve as a useful example of the non-standard complications of fulminant APS.

Primary antiphospholipid syndrome associated Liebman-Sachs endocarditis leading to diffuse alveolar hemorrhage: A case report

“…The most common of these manifestations include pulmonary hypertension and thromboembolic disease [1,2]. A less common initial manifestation of APS includes DAH which has been described in the literature as a non-criteria manifestation of APS [1,3,4]. A retrospective study demonstrated that DAH constituted 3.7% of systemic lupus erythematosus (SLE) admissions within a single institution [4].…”

“…A retrospective study demonstrated that DAH constituted 3.7% of systemic lupus erythematosus (SLE) admissions within a single institution [4]. As such, evaluation of DAH should include diagnostic testing not only for secondary APS but also primary APS as this may be a less common initial manifestation of these disease processes [[1], [2], [3],[5], [6], [7], [8], [9]]. Primary and secondary APS differ in that secondary APS has a concomitant diagnosis of another disease process, often SLE [8].…”

“…Although there can be overlap in clinical presentation among primary and secondary APS the incidence of valvular pathology is more common in those with secondary APS [10]. This form of marantic endocarditis is most commonly associated with mitral valve regurgitation thought to be the result of fibrin, platelet, leukocytes and plasma cells being deposited on the valves themselves [1,3,8]. In the case of mitral regurgitation, the flow of blood can be directed towards the right upper pulmonary vein leading to unilateral pulmonary edema and DAH [11].…”

“…Thrombotic events remain a hallmark of APS; among those presenting with DAH, multiple causes exist due to the presence of both inflammatory and noninflammatory states [9]. Case reports have demonstrated the etiology of DAH among small populations of those diagnosed with primary APS to be that of capillaritis [[3], [4], [5],9]. Inflammation leading to capillaritis is thought to be the result of direct vessel damage from the inflammation itself or because of immune complex deposition [9].…”

Antiphospholipid antibodies-associated diffuse alveolar hemorrhage

“…The available literature is limited to case reports and small case series [9][10][11][12][13][14][15][16][17]. It is also unclear whether the reported association is pathogenetically associated to aPL or PAPS.…”