Systemic lupus erythematosus with intracerebral hematoma due to decreased factor XIII activity: A rare association

Dabadghao, Varsha S; Singh, Vibha; Meena, Babu Lal; Khichar, Satyendra

doi:10.4103/0971-9903.117792

Cited by 1 publication

(2 citation statements)

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“…On review of literature there were very few case reports of intracerebral haemorrhage due to factor XIII deficiency, reported in already diagnosed patients with SLE. Usually acquired Factor XIII deficiency occurs a few years after the diagnosis of autoimmune disease (8). In our case it was the initial presentation of the diagnosis of SLE.…”

Section: Discussionmentioning

confidence: 61%

“…Cyclosporin, mycophenolate mofetil and other drugs can be given as second line treatment (9). It has been reported that a lupus patient with associated factor XIII deficiency who developed intracerebral haemorrhage was successfully treated with cyclophosphamide without any surgical evacuation as in the index case where the patient was successfully treated with glucocorticoids (8).…”

Section: Discussionmentioning

confidence: 99%

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Systemic lupus erythematosus presenting as acquired factor XIII deficiency and spontaneous subdural haemorrhage

Kirubaharan

Samarasingha

Kankanamge

et al. 2023

Asian J. Internal Med.

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Factor XIII deficiency is a rare, potentially life-threatening bleeding disorder. While congenital disease is inherited in an autosomal recessive manner, acquired factor deficiency can be associated with autoimmune disease or malignancy. Clinical spectrum may range from mucocutaneous bleeding to intracerebral bleeding. This case describes a 21-year-old woman who initially presented with low grade fever and cervical lymphadenopathy. In two weeks' time she developed bilateral spontaneous subdural haemorrhages along with bilateral small and large joint arthritis. Investigations revealed an acquired factor XIII deficiency, positive antinuclear antibodies (ANA) titer and Coomb's positive autoimmune hemolytic anaemia and was subsequently diagnosed to have systemic lupus erythematosus.Intracerebral bleeding due to acquired factor XIII deficiency is a rare initial presentation of systemic lupus erythematosus in literature.

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Section: Discussionmentioning

confidence: 61%

Section: Discussionmentioning

confidence: 99%