scholarly journals The Increased Frequency of Familial Mediterranean Fever in Patients Diagnosed with Immunoglobulin a Vasculitis (Henoch-Schönlein Purpura)

2018 ◽  
Vol 16 (1) ◽  
pp. 35-46
Author(s):  
Hafize Emine Sönmez ◽  
Ezgi Deniz Batu ◽  
Yelda Bilginer
Keyword(s):  
Familial Mediterranean Fever ◽  
Immunoglobulin A ◽  
Henoch Schonlein Purpura ◽  
Mediterranean Fever ◽  
Schonlein Purpura ◽  
Immunoglobulin A Vasculitis ◽  
Henoch Schönlein Purpura

scholarly journals Case of Gut Necrosis in Adult-Onset Immunoglobulin A Vasculitis (Henoch-Schönlein Purpura)

2020 ◽  
Vol 8 ◽  
pp. 232470962092556 ◽  
Author(s):  
Amanda S. Weissman ◽  
Viral Sanjay Patel ◽  
Omar Mushfiq
Keyword(s):  
Abdominal Pain ◽  
Renal Disease ◽  
Renal Involvement ◽  
Immunoglobulin A ◽  
Glucocorticoid Therapy ◽  
Acute Glomerulonephritis ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Immunoglobulin A Vasculitis ◽  
Henoch Schönlein Purpura

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.

Penile involvement of immunoglobulin a vasculitis/Henoch-Schönlein purpura

2021 ◽  
Author(s):  
Ummusen Kaya Akca ◽  
Ezgi Deniz Batu ◽  
Oguzhan Serin ◽  
Omer Faruk Ipek ◽  
Orkun Aydin ◽  
...  
Keyword(s):  
Immunoglobulin A ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Immunoglobulin A Vasculitis ◽  
Henoch Schönlein Purpura
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