CLINICAL QUIZ

An unusual cause of acute abdomen in an adolescentwith concurrent disease: Answers

Yasar Kandur & Sevcan A. Bakkaloglu & Ipek Isik Gonul &Idil Yenicesu & Koray Akkan & Buket Dalgic

Received: 17 September 2013 /Revised: 19 September 2013 /Accepted: 25 September 2013# IPNA 2013

Keywords Acute abdomen . Diabetes . Celiac disease .

Polyarteritis nodosa

Answers

1- Angiographic findings showing occlusion of a middle-sized artery and tissue biopsy findings demonstrating nec-rotizing non-granulomatous inflammation of the wall of amiddle-sized artery confirm the diagnosis of polyarteritisnodosa (PAN)

2- The mainstays of treatment for PAN are systemic steroidsand cyclophosphamide.We started with oral steroids (2mg/kg prednisolone) and pulse cyclophosphamide (CYX).After steroid tapering and the third dose of pulse CYX,she is symptom free and has normal acute-phase reactants.

This regimen was highly effective in the control of herdisease: the patient was discharged after 29 days withsteroid tapering (1.5 mg/kg/day) and a treatment regimenof steroids and monthly pulse CYX for 6 months. After thethird dose of CYX, she has been in complete remission.

Discussion

Polyarteritis nodosa (PAN) is a rare form of necrotizing vas-culitis of medium-sized arteries [1]. Disease severity is mainlybased on the extent and severity of vascular inflammation andnecrosis. Initial symptoms are generally nonspecific, includ-ing fever, malaise, arthralgia, abdominal pain, and weight loss.Although rare, acute or life-threatening clinical events maydevelop, such as ischemia/infarction of the kidney, intestinalischemia, cerebral or cardiovascular involvement, or deepnecrotic skin ulcers due to vasculitis and vascular stenosis[2]. The gastrointestinal tract is involved in approximatelyhalf of patients. However, acute surgical abdomen resultingfrom mesenteric infarction and bowel perforation as a firstmanifestation of PAN is very rare [3]. We herein report asuccessfully treated adolescent patient with PANwho present-ed with mesenteric arterial thrombosis and resultant intestinalischemia and perforation.

The particular interest of this case is the rare presentation ofPAN, characterized by intestinal ischemia and necrosis due toarterial thrombi, which resulted in perforation and abdominalemergency requiring surgical intervention. Past medical his-tory was also remarkable with concurrent diseases; type Idiabetes mellitus (DM) and celiac disease (CD). There areseveral potential explanations: first, DM has been reported inpatients with PAN possibly due to pancreatic involvement ofPAN [4]. However, our patient had DM 10 years before thediagnosis of PAN. Second, patients with type 1 DM are more

This refers to the article that can be found at http://dx.doi.org/10.1007/s00467-013-2648-8.

Y. Kandur (*) : S. A. BakkalogluDepartment of Pediatric Nephrology, School of Medicine, GaziUniversity, Ankara, Turkeye-mail: yaskan30@yahoo.com

I. I. GonulDepartment of Pathology, School of Medicine, Gazi University,Ankara, Turkey

I. YenicesuDepartment of Pediatric Hematology, School of Medicine, GaziUniversity, Ankara, Turkey

K. AkkanDepartment of Radiology, School of Medicine, Gazi University,Ankara, Turkey

B. DalgicDepartment of Pediatric Gastroenterology, School of Medicine, GaziUniversity, Ankara, Turkey

Pediatr NephrolDOI 10.1007/s00467-013-2649-7

prone to develop other autoimmune diseases, including CD[5]. In untreated CD, chronic activation of the immune systemand high levels of nonspecific organ antibodies, due toprolonged exposure to gluten, can induce the developmentof other autoimmune diseases [6].

Patients with immune-mediated diseases, including type IDM and PAN, have an increased risk of subsequent venousthrombotic events (VTE) [7]. Our patient had experienced twoprevious arterial thrombotic events: in the vertebral artery, whichcaused a mild clinical picture, and in the mesenteric arterialsystem with a severe disease. Since the association of a throm-botic mutation and PAN has not previously been described, wesuggest that the underlying genetic defect (heterozygous pro-thrombin 20210A mutation [8]), high levels of lipoprotein a,which are defined by the European Atherosclerosis Society(EAS) as a risk [9], and a vasculitic background contributed tothe development of repeated and life-threatening arterial throm-botic events in our patient. Although a similar adult case of PANwith gastrointestinal (GI) infarction and concurrent type I DMwas previously reported [10], our patient may be the first casehaving a thrombotic mutation, DM, CD, and PAN all together.

Transmural necrotizing inflammation in PAN weakens thearterial wall, causes stenosis, thrombosis, aneurysmal dilata-tion, and rupture. Impaired perfusion of the organs results inulceration, infarction, or ischemic atrophy [2]. Despite normalDoppler sonographic findings, we performed an angiographicexamination that showed occlusions of the ileal and jejunalbranches of the superior mesenteric artery (SMA). This dis-crepancy between two imaging techniques, which was previ-ously emphasized [11], highlights the superiority of selectiveangiography for detecting arterial lesions. In our case, wecould not detect any aneurysmal changes but complete occlu-sion in the intestinal branches of SMA progressing to tissuenecrosis and intestinal perforation. If ischemia is limited tothe gastrointestinal mucosa or submucosa, ulceration andbleeding may occur. Ischemic colitis presents with abdominalpain and diarrhea, sometimes bloody, often requiring surgery.Similar to the case of our patient, severe GI involvement inpediatric patients with PAN, as characterized by massivegastrointestinal necrosis with acute abdomen, perforation,massive bleeding and even a fatal course, has been published[12, 13]. The diagnosis was made by angiography in 55.5% ofthe patients with PAN, and through biopsies in the rest [14].Tissue biopsy may confirm the diagnosis of PAN, althoughinvolvement is segmental. While deep intestinal biopsies areoptimal for the diagnosis, they carry potential risks in acondition predisposed to infarction and perforation.

Conclusions

The present case is of interest for several reasons: (1) someindividuals may havemore than one immune-mediated disease;

(2) in cases with thrombotic events, vasculitic diseases, such asPAN, should also be considered; (3) further evaluation shouldbe undertaken for vasculitis despite the presence of geneticdefects that predispose for thrombosis; (4) selective angiogra-phy is the gold standard for the diagnosis of PAN; (5) promptdiagnosis and urgent and accurate treatment can be life savingeven in severe disease.

Statement

All human studies have been approved by the appropriateethics committee and have therefore been performed in accor-dance with the ethical standards laid down in the 1964Declaration of Helsinki. It should also be stated clearly in thetext that all persons gave their informed consent prior to theirinclusion in the study. Details thatmight disclose the identity ofthe subjects under study should be omitted. Masking of the eyeregion is not considered adequate protection.

Conflict of interest I accept the responsibility for the completion of thisdocument and attest to its validity on behalf of the co-authors. None of theauthors have any conflicts of interest.

Dr. Yasar Kandur 17.9.2013

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