A Case of Rapidly Progressive Nephrotic-Range Proteinuria in a Patient with Purpuric Rash

Introduction: IgA Vasculitis (IgAV) [formerly known as Henoch-Schonlein Purpura] is a systemic vasculitis involving IgA deposition in small vessels within various tissue types including the skin, joints, GI tract, and kidneys. It is often accompanied by a tetrad of signs/symptoms including palpable purpura, arthralgia, abdominal pain, and kidney disease. Case Description: A 77-year-old male with purpuric skin rash and skin biopsy-proven IgA vasculitis was referred by his dermatologist to the nephrology clinic after having found an acute kidney injury (serum creatinine: 1.5) along with hematuria and rapidly progressing proteinuria. Random total protein/creatinine ratio was 3.11 g/g, and 24-hour urine collection showed total protein 4.4 g. Renal biopsy was obtained showing Crescentic IgA Nephropathy. The patient was urgently admitted to the hospital and treated with pulse dose Methylprednisolone along with Cyclophosphamide. His kidney disease and proteinuria during his hospital stay stabilized on the immunosuppressive treatment, and he was discharged home one week later. Follow up in the nephrology clinic after 3 months of oral steroids showed stabilization of kidney function and proteinuria with creatinine level 1.4 mg/dL and urine protein/creatinine ratio 0.66 g/g. Discussion: IgA Vasculitis is not common among the adult population. The kidney involvement in IgA Vasculitis (termed IgAV nephritis) is more prevalent among adults than children, and long-term kidney prognosis tends to be worse in adults. Many patients present with only mild hematuria and proteinuria that is self-limited, but some patients may present with nephrotic range proteinuria. This case demonstrated the importance of close nephrology follow up in adults with new diagnosis of IgA Vasculitic rash as the initiation of immunosuppression can prevent the progression of nephrotic range proteinuria.