PW02-024-B - First report of AA amyloidosis in Blau syndrome

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PW02-024-B - First report of AA amyloidosis in Blau syndrome

Introduction Systemic AA amyloidosis is a life-threatening complication of different chronic infectious and inflammatory diseases. The deposition of amyloid fibrils derived from the serum amyloid A (SAA) protein represents its pathological hallmark. A long lasting and increased serum level of SAA is a prerequisite to its development. The group of inherited autoinflammatory diseases includes dif...

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Churg-Strauss syndrome associated with AA amyloidosis: a case report

Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/µL), positive p-ANCA, microscopic haematuria and proteinuria at 2g/day. A diagnosis of Churg-Strauss s...

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AA amyloidosis associated with hepatitis B.

We report a 13-year-old Indian boy with nephrotic syndrome caused by renal AA amyloidosis. Workup of the AA amyloidosis revealed chronic hepatitis B. Laser microdissection of the Congo-red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry confirmed the presence of serum amyloid A (SAA) protein and ruled out hereditary and familial amyloidosis. Further...

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Introduction Blau syndrome is a rare autoinflamattory granulomatous disease and inherited as autosomal dominant.The classical triad of Blau syndrome is granulomatous dermatitis, symmetric arthritis and recurrent uveitis. However, all of these findings may not be together in the patients. In the majority of patients, the disease is characterized by early onset that usually before 3-4 years of ag...

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ژورنال

عنوان ژورنال: Pediatric Rheumatology

سال: 2013

ISSN: 1546-0096

DOI: 10.1186/1546-0096-11-s1-a165