Systemic lupus erythematosus presenting with haemorrhagic manifestation

Abstract

A 26‐year‐old female presented with an episode of severe mucus membrane bleeding. Investigations revealed prolonged prothrombin time (PT), and partial thromboplastin time (PTT), normal thrombin time (TT) and reptilase time, thrombocytopenia, a positive test for lupus anticoagulant (LA), as well as anti‐cardiolipin antibodies (ACL). A toxicology screen for toxic drugs and coumadin was negative. Coagulation factor assays revealed low levels for factor II and XII. Low level inhibitor to factor II was demonstrated. Patient had a negative VDRL test and positive anti‐nuclear antibodies (ANA). The diagnosis of acquired hypoprothrombinaemia secondary to circulating inhibitor induced by LA was made, and then the patient was started on prednisone, which led to cessation of the bleeding and normalization of PT and PTT, as well as an increase of factor II and factor XII levels. A few months later, the patient developed arthralgia and alopecia, and antibodies against double‐stranded DNA were detected, and the diagnosis of systemic lupus erythematosis (SLE) was confirmed. The patient continued to have mild prolongation of PT and PTT while on a low dose of prednisone, but she had no bleeding symptoms. A computed tomography scan of the brain was carried out for unexplained central nervous system (CNS) symptoms, and it revealed mild hydrocephalus, which was thought to be part of the CNS manifestations of SLE. It was concluded that patients with SLE may present with haemostatic defects that are a result of either platelet‐related causes (quantitative or qualitative) or coagulation factor deficiency secondary to circulating inhibitor, or both, in the absence of other features of SLE which may appear later.

Diagnosis of lupus anticoagulant in the lupus anticoagulant‐hypoprothrombinemia syndrome: Report of two cases and review of the literature

“…On some occasions, it can present with hemorrhagic diathesis as a consequence of LA-HPS. Since its first descrip-tion in 1960 by Rapaport et al [9] in an 11-year-old girl with SLE and severe bleeding, the LA-HPS has been documented in the literature in 29 pediatric patients [1-3,10-23] and less frequently in adult patients [4,[24][25][26][27][28][29][30][31][32][33][34][35].…”

Hypovolemic shock as an initial presentation of pediatric systemic lupus erythematosus

“…Ayoub et al . reported a 26 year‐old woman who presented with an episode of mucus membrane bleeding, prolonged PT, and PTT, thrombocytopenia, a positive test for LA, as well as anticardiolipin antibodies 10 . The patient had a 3 month history of epistaxis, menorrhagia, and mild bleeding per rectum 10 .…”

“…reported a 26 year‐old woman who presented with an episode of mucus membrane bleeding, prolonged PT, and PTT, thrombocytopenia, a positive test for LA, as well as anticardiolipin antibodies 10 . The patient had a 3 month history of epistaxis, menorrhagia, and mild bleeding per rectum 10 . In previously reported SLE patients with hemorrhagic symptoms, mild or moderate bleeding might not lead to a life‐threatening condition, but massive bleeding such as occurred in the present patient would necessitate emergency intensive care.…”

Resolution of Hypoprothrombinemia‐Lupus Anticoagulant Syndrome (HLAS) after multidrug therapy with rituximab: a case report and review of the literature

“…In cases where the antibody persists, therapy has mainly consisted of immunosuppressive drugs. Corticosteroids remain the backbone of initial therapy [3,8,9,[12][13][14][15][17][18][19][20][21][22][23][24][25][26][27]. It is postulated that steroids decrease the clearance of prothrombin-antiprothrombin complexes [9].…”