Abstract
Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is an immune-mediated small vessel vasculitis characterized by palpable purpura, arthralgia, abdominal pain, and renal disease. It is primarily a childhood disease and usually resolves spontaneously with supportive therapy. Treatment of IgAV in adults is controversial with no clearly established guidelines. We report a rare case of IgAV in an adult male who developed gut necrosis and perforation while receiving glucocorticoid therapy for treatment of acute glomerulonephritis. A 44-year-old male was admitted with joint pain, leg swelling, mild abdominal pain, and a diffuse rash. Laboratory values revealed acute kidney injury with significant proteinuria and hematuria. The patient was started on glucocorticoid therapy for suspected IgAV nephritis, which was confirmed by kidney biopsy. Several days later, he complained of worsening abdominal pain. Imaging demonstrated bowel ischemia and perforation requiring multiple abdominal surgeries. The patient was critically ill in the intensive care unit with worsening renal failure requiring dialysis. He was discharged a month later after gradual recovery with stable but moderately impaired kidney function. IgAV is less common in adults; however, the disease is more severe with a higher risk of long-term complications. Adult patients with renal involvement may benefit from glucocorticoid therapy in preventing progression to end-stage renal disease. However, glucocorticoids may mask the symptoms of abdominal complications like gut necrosis and perforation causing delay in diagnosis and treatment. Therefore, vigilance to detect early signs of gut ischemia is imperative when treating an adult case of IgAV nephritis with glucocorticoids.
Highlights
Immunoglobulin A vasculitis (IgAV), previously referred to as Henoch-Schönlein purpura, is the most common systemic vasculitis in children
IgAV in adults tends to be a more severe disease than it is in children and carries an increased risk of progressive renal disease and end-stage renal disease (ESRD).[13]
Otherwise known as IgAV nephritis (IgAVN), is the most serious manifestation of IgAV since it is the only organ linked to long-term morbidity and mortality in both childhood-onset and adult-onset disease.[14]
Summary
Immunoglobulin A vasculitis (IgAV), previously referred to as Henoch-Schönlein purpura, is the most common systemic vasculitis in children. IgAV is characterized by a tetrad of clinical manifestations that includes palpable purpura, arthralgia, renal disease, and abdominal pain.[5] Diagnosis is confirmed by performing a biopsy demonstrating deposition of IgA in affected organs. An exception is adults with renal disease, who have a much higher incidence of severe renal failure and progression to end-stage renal disease (ESRD).[6,7,8] For the majority of patients, treatment is generally supportive including bed rest, hydration, and pain control with nonsteroidal anti-inflammatory drugs.
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