Multisystem failure due to three coexisting autoimmune diseases.
Systemic lupus erythematosis (SLE) is a potentially fatal, autoimmune disease, which can affect different organs and can present with protean clinical manifestations. It may be associated with many other autoimmune conditions and two rare such conditions are myelofibrosis and acquired haemophilia. Autoimmune myelofibrosis is a bone marrow disorder characterized by pancytopenia, which can occur in conjunction with the presenting features, or an exacerbation of previously established SLE. Acquired haemophilia is another rare disorder of haemostasis, which can be life threatening without prompt and appropriate treatment. The management of these different conditions in itself poses a difficult problem but when the three conditions present simultaneously in the same individual, the accurate diagnosis and indeed the appropriate management becomes extremely challenging. This report describes a young woman who presented with pancytopenia secondary to myelofibrosis and panserositis with no identifiable precipitating factors. Her condition deteriorated rapidly and she required intensive care support for respiratory failure and renal impairment. A presumed diagnosis of SLE was considered and treatment was initiated which improved and stabilised her condition. However, she developed bleeding complications from acquired haemophilia which required further specialist intervention. Multidisciplinary management of the patient helped in the resolution of the complications and stabilisation of her autoimmune conditions. This report should make physicians aware of the rare presentations of SLE and its complex management.
Pillai
Arvind
A
Department of Haematology, Royal Liverpool University Hospital, Liverpool, UK.
Gautam
Manish
M
Williamson
Helen
H
Martlew
Vanessa
V
Nash
John
J
Thachil
Jecko
J
D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications* / Q000628:therapy; D005260:Female; D006467:Hemophilia A / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D009102:Multiple Organ Failure / Q000209:etiology* / Q000628:therapy; D010198:Pancytopenia / Q000209:etiology; D055728:Primary Myelofibrosis / Q000150:complications; D012700:Serositis / Q000209:etiology
D007166:Immunosuppressive Agents