PMC3558347_01
Purpura. (A) Diffuse small palpable purpura on the lower extremities. (B) Magnified view.
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Physical examination revealed pretibial pitting edema and diffuse small palpable purpuras on both lower extremities (Figure 1A, B) and on the lower abdomen.
Computed tomography of the abdomen. (A) Computed tomography of the abdomen performed on admission, showing diffuse thickening of the bowel wall with target signs in the small intestine and ascites (arrow). (B) Computed tomography of the abdomen performed on day 9, showing severe edematous and dilatational changes in the small intestine and ascites (arrow) before treatment with steroids and cyclophosphamide. (C) Computed tomography of the abdomen performed on day 29 before treatment, showing ascites (arrows) that remained after treatment with steroids and cyclophosphamide. However, the severe edematous changes in the small intestine were slightly improved relative to day 9.
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Computed tomography of the patient's abdomen demonstrated diffuse thickening of the bowel wall, with target signs in the small intestine and ascites (Figure 2A). College of Rheumatology criteria. Computed tomography of the abdomen performed on day 9 showed severe edematous and dilatational changes in the small intestine and ascites before treatment with steroids and cyclophosphamide (Figure 2B). Twenty-nine days after treatment began, the ascites remained, and there were severe edematous changes in the small intestine (Figure 2C).
Colonoscopy findings. Colonoscopy images showing patchy erythema in the sigmoid colon (A) and rectum (B) on day 4, before treatment began. (C, D) Colonoscopy images showing fresh bleeding in the ascending sigmoid colon on day 29, after treatment with steroids and cyclophosphamide.
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However, there were patchy areas of erythema in the sigmoid colon (Figure 3A) and rectum (Figure 3B). Colonoscopy confirmed frank bleeding from the ascending sigmoid colon (Figure 3C, Dc, d).
Treatment and clinical course. Top panel, doses and schedules of drug therapies. Middle panels, clinical findings. Abdominal pain and melena appeared after improvement of purpura and before treatment with steroid and immunosuppressants. The patient's melena and proteinuria did not improve after treatment with steroids, cyclophosphamide, infusion of RCC-LR (red cell concentrates - leukocytes reduced), and intravenous PGE1 (prostaglandin E1). Bottom panels, laboratory findings. Cre, creatinine; CRP, C-reactive protein; Hb, hemoglobin.
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After intravenous treatment with Factor XIII concentrate (20 mL/day for 3 consecutive days) and prednisolone sodium succinate (40 mg/day for 13 consecutive days; see Figure 4 for dosage schedules of all drugs), the skin lesions and abdominal pain disappeared completely. However, gastrointestinal hemorrhage and mild proteinuria remained (Figure 4) and did not improve upon treatment with intravenous methylprednisolone (pulse dose of 1000 mg/day for 3 consecutive days) and intravenous cyclophosphamide (800 mg on 1 day).
Supramesenteric artery angiography. (A) Mesenteric angiogram showing absence of bleeding points and vascular occlusion, but presence of narrowing and irregularity of major branches of the superior mesenteric artery. (B) Mesenteric angiogram taken on day 30, showing blood flow and reduced vascular narrowing after catheter injection of 10 mug of PGE1 and 20 mg of prednisolone sodium succinate into the superior mesenteric artery.
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However, angiography revealed narrowing and irregularities in the major branches of the superior mesenteric artery and absence of blood flow, indicative of NOMI (Figure 5Aa). On day 30, blood flow in the superior mesenteric artery increased and vascular narrowing improved after catheter injection of 10 mug of PGE1 and 20 mg of prednisolone sodium succinate into the artery (Figure 5B).
Autopsy findings. Biopsy samples were fixed and stained with hematoxylin and eosin. Histopathology of the jejunal submucosa shows (A) an exposed small vessel (arrow) (x40) and (B) exposed vessel and ruptured elastic laminae in the tunica externa of that vessel (x100). Histopathology of the tunica media of the superior mesenteric artery shows (C) no active vasculitis or thrombosis at autopsy (x40), but (D) edematous change (x200).
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Histopathologic examination of the submucosa of the jejunum revealed an exposed small vessel (Figure 6A), as well as obliteration of small vessels and ruptured elastic laminae in the tunica externa of those vessels (Figure 6B). There was no evidence of active vasculitis or thrombosis in the tunica media of the superior mesenteric artery (Figure 6C), although there were edematous changes (Figure 6D).