A PURPURIC PREDICAMENT: IGA VASCULITIS IN END STAGE KIDNEY DISEASE

Dr Danial Mahmood1, Dr Lucy McMullen1,3, Dr Aviv Pudipeddi1,2, Dr Sandhya Limaye1,2, Dr Tai Anh Phan1,2, Dr Renee Chan1, Dr Shaundeep Sen1,2

1Concord Repatriation General Hospital, Sydney, Australia, 2Concord Clinical School, Faculty of Medicine and Health, University of Sydney, Sydney, Australia, 3St George Clinical School, Faculty of Medicine and Health, University of New South Wales, Sydney, Australia

Biography:

Bio to come.

Background:

IgA vasculitis (IgAV) is a common manifestation of IgA mediated disease most often seen in children, presenting with the triad of a purpuric rash without haematological abnormality, arthritis, abdominal pain and kidney involvement. IgAV is rarely observed in adults with end stage kidney disease (ESKD), and there are no international guidelines on criteria for management in this setting.

Case presentation:

We present a case of a 68-year-old man with end stage kidney disease (presumed secondary to diabetic nephropathy) on maintenance intermittent haemodialysis. Two days after inpatient admission for intravenous antibiotics for cellulitis, he re-presented with glossitis and a purpuric rash, closely followed by severe hypertension, macroscopic haematuria, and colitis.

IgAV diagnosis was confirmed by clinical presentation, raised serum IgA (6.03 g/L), and invasive testing. Skin biopsy confirmed leukocytoclastic vasculitis with IgG and IgA staining collagen fibres. Sigmoidoscopy showed multiple inflammatory areas, with biopsy demonstrating mucosal inflammation without granulomas or blood vessel involvement. Resolution of persisting severe abdominal symptoms was achieved through a short course of oral corticosteroids.

Conclusion:

While the incidence of IgAV in ESKD is low, diagnosis and management can be challenging due to a paucity of evidence. Our case demonstrates an interesting constellation of interrelated disease associations and possible triggers, including allergy (glossitis presumed due to use of angiotensin-converting enzyme inhibitor) and infection (prior presentation with cellulitis). Overlap of IgAV with renal involvement caused us to question the presumed diagnosis of diabetic nephropathy, though given dialysis dependence a kidney biopsy was not performed.

 

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