Rahaf Z Attar
King Abdulaziz University, Saudi Arabia
Title: SLE patient with an initial presentation of hus triggered by psgn (case report and review)
Biography
Biography: Rahaf Z Attar
Abstract
Background:
Systemic lupus erythematosus is an autoimmune multisystemic disease that can be present in myriad of ways. Thrombotic microangiopathy is a rare complication of SLE. Even more rare if it was the initial presentation of the disease. Infection has been suggested to be one of the triggers for such an event. There are few cases reported on SLE patients initially presenting as HUS. Other few cases on HUS triggered by a streptococcal infection.
Methods:
We describe a case of a 9 year old boy who had a history of pharyngitis, two weeks later he presented to the hospital with symptoms of severe anemia, hematuria and abnormal renal function. Investigations showed positive ASO , ANA , dsDNA antibodies.
Results
Biopsy revealed a picture of thrombotic microangiopathy in addition to a diffuse thickening of the glomerular basement membrane with membrnoproliferative pattern of proliferation. Diagnosis of SLE and aHUS was made. The patient was improved after the treatment with prednisone and cyclophosphamide. No plasma exchange was required.
Conclusions
aHUS is a type of thrombotic microangiopathy that might present as a vascular complication of SLE. infection is not only an important risk factor for SLE exacerbation but also a common trigger for thrombotic microangiopathic events in these patients. There are several cases reported in the literature of SLE patients first presenting as aHUS. However, the occurrence of aHUS triggered by streptococcal infection as the first presentation in an SLE patient haven't been reported to the extent of our knowledge. The outcome is generally favorable.