A CASE OF REFRACTORY IgA VASCULITIS COMLICATED WITH GASTROINTESTINAL VENOUS VASCULITIS SIMILAR TO Behçet's DISEASE IN WHICH INFLIXIMAB WAS EFFECTIVE

 

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A CASE OF REFRACTORY IgA VASCULITIS COMLICATED WITH GASTROINTESTINAL VENOUS VASCULITIS SIMILAR TO Behçet's DISEASE IN WHICH INFLIXIMAB WAS EFFECTIVE

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Sakiko
Takahashi
Sakiko Takahashi saki.st2001@gmail.com Toranomon Hospital Nephrology Center and Okinaka Memorial Institute for Medical Research Tokyo Japan *
Eiko Hasegawa eiko-hase@hotmail.co.jp Toranomon Hospital Nephrology Center and Okinaka Memorial Institute for Medical Research Tokyo Japan -
Akira Tanimizu ravine.hermit0424@gmail.com Toranomon Hospital Nephrology Center and Okinaka Memorial Institute for Medical Research Tokyo Japan -
Akinari Sekine akinari-s@toranomon.gr.jp Toranomon Hospital Nephrology Center and Okinaka Memorial Institute for Medical Research Tokyo Japan -
Nobukazu Hayashi hayashi@toranomon.gr.jp Toranomon Hospital Department of Dermatology Tokyo Japan -
Yutaka Mitsunaga mit.yutaka@gmail.com Toranomon Hospital Department of Gastroenterology Tokyo Japan -
Kei Matsui matsui3514@gmail.com Toranomon Hospital Department of Gastroenterology Tokyo Japan -
Masanori Ueno ueno@toranomon.gr.jp Toranomon Hospital Department of Gastrointestinal surgery Tokyo Japan -
Kei Kono k.kono0317@gmail.com Toranomon Hospital Department of Pathology Tokyo Japan -
Kenichi Ohashi kohashi.pth1@tmd.ac.jp Institute of Science Tokyo Department of Human Pathology Tokyo Japan -
Tamiko Takemura tamikobyori@gmail.com Kanagawa Cardiovascular and Respiratory Center Department of Pathology Kanagawa Japan -
Kenji Notohara notohara@kchnet.or.jp Kurashiki Central Hospital Department of Anatomic Pathology Kurashiki Japan -
Takehiko Wada takewada@gmail.com Toranomon Hospital Nephrology Center and Okinaka Memorial Institute for Medical Research Tokyo Japan -
Naoki Sawa naokis@toranomon.gr.jp Toranomon Hospital Nephrology Center and Okinaka Memorial Institute for Medical Research Tokyo Japan -
Yoshifumi Ubara ubara@toranomon.gr.jp Toranomon Hospital Nephrology Center and Okinaka Memorial Institute for Medical Research Tokyo Japan -

We report the diagnosis and treatment of a 22-year-old woman who visited our hospital complaining of facial erythema, abdominal discomfort, and subsequent vomiting.

On admission, miliary purpura was noted on the limbs and face, along with polyarthralgia. Endoscopy revealed erosions with redness in the duodenum, and a biopsy was performed. A diagnosis of venous vasculitis was made, with hemorrhage and fibrin deposition in the submucosa and significant neutrophil infiltration. A skin biopsy revealed leukocytoclastic vasculitis with fibrin deposition throughout the dermis. Treatment with 1 mg/kg prednisolone was initiated, but the condition was refractory. One month after the onset of symptoms, newly proteinuria (1 g/g Cre) and urinary occult blood were noted, and a renal biopsy was performed. Glomerular lesions with IgA deposition, fibrin deposition and cellular crescent formation were observed, leading to a diagnosis of severe IgA vasculitis.

Steroid pulse therapy was initiated in combination with azathioprine. Thereafter, azathioprine was replaced with mycophenolate mofetil, and colchicine was added to the regimen. Five months after the onset of symptoms, severe lower abdominal pain suddenly appeared. A diagnosis of ileocecal perforation was made, and an emergency laparoscopic ileocecal resection was performed. Pathological examination revealed venous vasculitis in the ulcer at the perforation site, consistent with gastrointestinal lesions of intestinal Behçet's disease. Although no oral aphthous lesions or uveitis were present, the patient received infliximab therapy as described for Behçet's disease. Urinary protein and occult blood rapidly disappeared, and abdominal pain also improved. The patient's progress is favorable with continued infliximab administration.

We report a case of IgA vasculitis presenting with steroid-resistant gastrointestinal lesions. Venous vasculitis similar to Behçet's disease was diagnosed as the primary pathology, and TNF therapy, which is effective for Behçet's disease, was effective for this patient. Although IgA vasculitis is described as inflammation of small vessels in the skin, kidneys, and intestines, no detailed explanation was given as to which part of the blood vessels (arterial or venous) it corresponds to. Our findings suggest that venous vasculitis plays a key role in steroid resistance. Thus, considering IgA vasculitis as an autoinflammatory disease, anti-cytokine therapies—particularly TNF inhibitors—may offer a viable treatment option for steroid-resistant IgA vasculitis.

Kewords