Shah Raj, Ramakrishnan Madhuri, Vollmar Alexis, Harrell Amanda, Van Trump Richard, Masoud Amgad
Department of Internal Medicine, University of Missouri - Kansas City.
Medical Student, University of Missouri.
Cureus. 2017 Mar 9;9(3):e1088. doi: 10.7759/cureus.1088.
Henoch-Schönlein purpura (HSP) is typically seen as a self-limiting disease in children, but can present more severely in adults, especially when there is renal involvement. Management of HSP in adults also remains a controversial topic with very few studies evaluating available therapies. In this case, HSP presenting as a combination of severe gastrointestinal involvement and a rapid decline in renal function in an adult patient directed our therapy. The patient was a 48-year-old Caucasian male with no known past medical history, who presented with a combination of purpuric rash over the lower extremities, severe abdominal pain with upper gastrointestinal bleeding and a rapidly increasing serum creatinine, with hematuria. He initially underwent a skin biopsy, along with investigation for other possible causes, including autoimmune and infectious etiologies, which were negative. He was started on therapy for presumed HSP with intravenous methylprednisolone. The skin biopsy, however, was not conclusive, and the patient had no improvement in his clinical status. He then underwent a kidney biopsy that was consistent with HSP nephritis (immunoglobulin A (IgA) predominant glomerulonephritis with crescents), and esophagogastroduodenoscopy (EGD) that showed mucosal inflammation, ulcerations, and stigmata of bleeding-findings that were consistent with ischemia. Cyclophosphamide was added to the regimen at this time. However, he had worsening abdominal pain, continued gastrointestinal bleeding, now with hematochezia, and also worsening renal function that required dialysis. Plasmapheresis was then initiated on days alternating with dialysis. This resulted in the improvement of his gastrointestinal symptoms, but no recovery was seen of his renal function, and the patient required outpatient dialysis. This case report exhibits the unique presentation of severe gastrointestinal (GI) manifestations and rapid progression to renal failure in an adult patient with partial resolution of his severe manifestation after therapy was escalated as above. There was no established protocol that guided this therapy, which reflects the need for more studies on adult HSP.
过敏性紫癜（HSP）在儿童中通常被视为一种自限性疾病，但在成人中可能表现得更为严重，尤其是在出现肾脏受累时。成人HSP的管理仍然是一个有争议的话题，很少有研究评估现有的治疗方法。在本病例中，一名成年患者的HSP表现为严重的胃肠道受累和肾功能迅速下降，这指导了我们的治疗。患者为一名48岁的白种男性，既往无已知病史，表现为下肢紫癜性皮疹、伴有上消化道出血的严重腹痛以及血清肌酐迅速升高和血尿。他最初接受了皮肤活检，并对其他可能的病因进行了调查，包括自身免疫和感染性病因，结果均为阴性。他开始接受静脉注射甲泼尼龙治疗疑似HSP。然而，皮肤活检结果不明确，患者的临床状况没有改善。随后他接受了肾脏活检，结果符合HSP肾炎（以免疫球蛋白A（IgA）为主的新月体性肾小球肾炎），食管胃十二指肠镜检查（EGD）显示黏膜炎症、溃疡和出血迹象，这些发现与缺血一致。此时在治疗方案中加入了环磷酰胺。然而，他的腹痛加重，胃肠道持续出血，现在出现便血，肾功能也恶化，需要透析。然后在与透析交替的日子里开始进行血浆置换。这导致了他胃肠道症状的改善，但肾功能没有恢复，患者需要门诊透析。本病例报告展示了一名成年患者严重胃肠道（GI）表现的独特呈现以及迅速进展为肾衰竭的情况，在如上升级治疗后其严重表现部分得到缓解。没有既定的方案指导这种治疗，这反映了对成人HSP进行更多研究的必要性。
