Systemic lupus erythematosus presenting with haemorrhagic manifestation

A 26-year-old female presented with an episode of severe mucus membrane ble eding. Investigations revealed prolonged prothrombin time (PT), and partial thromboplastin time (PTT), normal thrombin time (TT) and reptilase time, t hrombocytopenia, a positive test for lupus anticoagulant (LA), as well as a nti-cardiolipin antibodies (ACL). A toxicology screen for toxic drugs and c oumadin was negative. Coagulation factor assays revealed low levels for fac tor II and XII. Low level inhibitor to factor II was demonstrated. Patient had a negative VDRL test and positive anti-nuclear antibodies (ANA). The di agnosis of acquired hypoprothrombinaemia secondary to circulating inhibitor induced by LA was made, and then the patient was started on prednisone, wh ich led to cessation of the bleeding and normalization of PT and PTT, as we ll as an increase of factor II and factor XII levels. A few months later, t he patient developed arthralgia and alopecia, and antibodies against double -stranded DNA were detected, and the diagnosis of systemic lupus erythemato sis (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 symp toms. A computed tomography scan of the brain was carried out for unexplain ed central nervous system (CNS) symptoms, and it revealed mild hydrocephalu s, which was thought to be part of the CNS manifestations of SLE. It was co ncluded that patients with SLE may present with haemostatic defects that ar e a result of either platelet-related causes (quantitative or qualitative) or coagulation factor deficiency secondary to circulating inhibitor, or bot h, in the absence of other features of SLE which may appear later.