An adult patient with Henoch-Schönlein purpura and non-occlusive mesenteric ischemia
© Oshikata et al.; licensee BioMed Central Ltd. 2013
Received: 17 August 2012
Accepted: 18 January 2013
Published: 23 January 2013
Onset of Henoch-Schönlein purpura (HSP) in middle age is uncommon, and adults with renal or gastrointestinal involvement present with more severe disease than do similar pediatric patients.
We present the case of a 69-year-old male with HSP who, after treatment with steroids, cyclophosphamide, and continuous intravenous prostaglandin E1 (PGE1), died as a result of severe gastrointestinal involvement with non-occlusive mesenteric ischemia (NOMI). Vascular narrowing associated with the NOMI improved after catheter injection of PGE1 and prednisolone, but the patient died of bleeding from an exposed small vessel. At autopsy there was no active vasculitis in the jejunal submucosa.
Treatment with PGE1 and prednisolone might improve small-vessel vasculitis associated with NOMI.
KeywordsHenoch-Schönlein purpura Intervention Non-occlusive mesenteric ischemia Small vessel vasculitis
Henoch-Schönlein purpura (HSP) is characterized by a leukocytoclastic vasculitis involving the small vessels, with deposition of immune complexes that contain IgA [1, 2]. Clinical signs include purpura, arthralgia, glomerulonephritis, and gastrointestinal involvement . HSP occurs primarily in children . It is uncommon in people over the age of 40, and little is known about its natural history in this population . The prognosis for patients with childhood-onset HSP is good. However, the clinical presentation of HSP in adults is severe and the clinical outcome relatively poor . In particular, deaths have been reported in cases of adult HSP with severe gastrointestinal involvement [7, 8].
Non-occlusive mesenteric ischemia (NOMI) is defined as acute mesenteric ischemia through hypoperfusion caused by ongoing splanchnic vasoconstriction, without demonstrable occlusion of the mesenteric vasculature . NOMI is a life-threatening vascular emergency that requires early diagnosis and intervention to adequately restore mesenteric blood flow and prevent bowel necrosis and patient death. Risk factors for NOMI include hypovolemia, hypotension, low cardiac output status, renal or hepatic disease, cardiac surgery, and administration of α-adrenergic agonists, digoxin, or β-receptor blocking agents . There are no published reports of NOMI associated with HSP.
We present a fatal case of HSP in an adult patient who had severe gastrointestinal involvement with NOMI. After treatment with steroids, cyclophosphamide, intravenous steroids, prostaglandin E1 (PGE1), and continuous intravenous papaverine hydrochloride, the ischemic change caused by small-vessel vasculitis of the small intestine improved. Unfortunately, the patient had a poor prognosis owing to bleeding from an exposed small vessel, although at autopsy he had no apparent active vasculitis in the jejunal submucosa.
A 69-year-old Japanese male presented with a history of bronchial asthma from age 61 and hypertension from age 50. He had been treated with inhaled glucocorticosteroids, long-acting inhaled β2-agonists, leukotriene modifiers, methylxanthines, and antihypertensives. He was an ex-smoker with a Brinkman Index of 1200. He had noticed purpura appearing in both lower extremities without any preceding infection, including upper respiratory tract infection. He then developed, without fever, edema in both lower extremities and arthralgia in the right foot. Four days after the patient had first noticed symptoms, the purpura gradually improved. However, he began suffering from abdominal pain, diarrhea, edema of the upper extremities, and oliguria.
Although HSP is typically a disease of children, adult cases can occur and present as more severe disease. Serious complications related to gastrointestinal involvement include intussusception, infarction, and perforation [7, 11, 12]. HSP patients who are older than 60 years at onset and who have renal or gastrointestinal involvement have a poor prognosis [5–8, 13]. Some patients with gastrointestinal involvement die despite treatment with steroids and immunosuppressants [8, 13]. Among adult patients with HSP, 24.1% have initial onset with gastrointestinal involvement before the cutaneous rash , and those manifestations may be helpful for early diagnosis and for selecting appropriate management strategies .
Few reports of NOMI associated with small-vessel vasculitis have been published. There has been one case report of vasculitic lesions that resulted in dialysis-related hypotension and NOMI, but this was associated with giant cell arteritis . NOMI in our patient might have been caused by active vasculitis, but it appeared not to have been caused by hypotension or hypovolemia, and the patient did not receive drugs such as α-adrenergic agonists, digoxin, or β-receptor blocking agents, which are known to precipitate this condition . NOMI has a high mortality rate, and early diagnosis and treatment are important for improving survival . Early treatment of NOMI patients with continuous intravenous PGE1 increases survival rates [15, 16]. Abdominal pain and melena in our patient appeared after the purpura improved and before treatment with steroids or immunosuppressants. The patient’s melena and renal dysfunction did not improve after treatment with steroids, cyclophosphamide, and intravenous PGE1 and steroids. At autopsy, an exposed small vessel was identified, but there was no evidence of active vasculitis in the submucosa of the jejunum. We suspect that the treatment for NOMI improved vasodilatation in the submucosa of the small intestine, thus improving the distribution of the locally injected steroid or the ongoing systemic steroid and immunosupressant to the submucosa of the jejunum. We considered that exposure of the small vessel, possibly as a result of the remodeling process in the submucosa, was the cause of death.
Treatment of NOMI results in vasodilatation, thus resulting in more effective treatment of the active small-vessel vasculitis associated with HSP.
Written informed consent was obtained from the kin of the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Non-occlusive mesenteric ischemia
Red cell concentrates – leukocytes reduced.
The authors thank Mizuho Yamamoto (Department of Dermatology, National Hospital Organization Sagamihara National Hospital) and Yasuyo Takeshita (Department of Nephrology, National Hospital Organization Sagamihara National Hospital) for their clinical support.
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