A Renal Transplant Recipient With Delayed Gastric Emptying in Amyloidosis Due to Familial Mediterranean Fever Improved With Erythromycin: A Case Report F. Saglam, A. Celik, C. Cavdar, A. Sifil, K. Atila, G.Ç. Kaya, S. Bora, H. Gulay, and T. Camsari ABSTRACT Patients with systemic amyloidosis often have symptoms related to impared gastrointestinal motility due to delayed gastric emptying, which results from autonomic nerve or smooth muscle infiltration with amyloid. There is no current report about gastric delaying secondary to amyloidosis due to familial Mediterranean fever. In this report, we have described a renal transplant recipient with delayed gastric emptying secondary to amyloidosis due to familial Mediterranean fever, which improved with erithromycin treatment.
A
MYLOIDOSIS RESULTS from the deposition of insoluble, fibrous amyloid proteins, nearly always in extracellular spaces of organs and tissues.1 Renal involvement usually occurs in primary or secondary amyloidosis. Renal transplantation is an accepted treatment of end-stage renal failure due to amyloidosis.2 Patients with systemic amyloidosis often have symptoms related to impared gastrointestinal motility due to delayed gastric emptying, which results from autonomic nerve or smooth muscle infiltration with amyloid. There is no current report about gastric delaying secondary to amyloidosis due to familial Mediterranean fever (FMF). In this report, we describe a renal transplant recipient with delayed gastric emptying secondary to amyloidosis due to FMF, which improved with erithromycin treatment. CASE REPORT A 27-year-old male patient underwent a living related kidney transplantation for end-stage renal disease (ESRD) due to amyloidosis secondary to FMF. Seven years after transplantation, he was admitted with the complaints of gastroparesis: nausea, vomiting, and weight loss. On examination, his blood pressure was 140/80 mm Hg and pulse rate 78 beats/min. He displayed epigastric tenderness but other physical findings were normal. Laboratory findings were normal except the serum creatinine level of 2 mg/dL. His current immunsuppresive regimen included prednisolone (5 mg/d), mycophenolate mofetil (1500 mg/d), and cyclosporine (200 mg/d). He was also treated with colchicine (1 mg/d), kinapril (10 mg/d), and omeprazole (20 mg/d). On upper gastrointestinal endoscopy examination, he was found to have retention of food even after a 12-hour fast. Gastric amyloidosis was established by biopsy. Congo red staining identi0041-1345/08/$–see front matter doi:10.1016/j.transproceed.2007.11.012 308
fied systemic secondary (AA) amyloid fibrils in the media of gastric blood vessels. With these findings a 99m-technetium (99mTc)labeled meal scintigraphy was performed to record gastric emptying. The half time (T50) of the emptying phase was over 194 minutes (Fig 1). The patient was treated with erithromycin (2 ⫻ 250 mg/d, orally). After only after a few days his symptoms improved, and after a month he had no complaint. A control endoscopy performed after 2 months of treatment revealed an edematous bulbus without any food retention. The control scintigraphy revealed a T50 of the emptying phase to be 100 minutes (Fig 2) suggesting that erythromycin treatment quantitatively enhanced gastric emptying.
DISCUSSION
FMF is one of several disorders classified as a hereditary periodic fever syndrome. The prognosis for patients with FMF is mainly determined by the presence of AA amyloidosis, which is caused by deposition of serum amyloid A (SAA).3 SAA occurs commonly in the kidneys, liver, adrenals, intestine, spleen, and joints as well as less commonly in the heart, lungs, testes, and thyroid. Congo red is helpful to determine the amyloid component as AA rather than AL type. Scintigraphy with radiolabeled serum amyloid P component could support the diagnosis of amyloidosis in patients with negative histological studies, distinguishing a From the Departments of Nephrology (F.S., A.C., C.C., A.S., T.C.), General Surgery (K.A., S.B., H.G.), and Nuclear Medicine (G.Ç.K.), Dokuz Eylul University, School of Medicine, Balcova, Izmir. Address reprint requests to Funda Saglam, MD, Dokuz Eylul University School of Medicine, Department of Nephrology, Balcova, Izmir. E-mail:
[email protected] © 2008 by Elsevier Inc. All rights reserved. 360 Park Avenue South, New York, NY 10010-1710 Transplantation Proceedings, 40, 308 –309 (2008)
DELAYED GASTRIC EMPTYING IN AMYLOIDOSIS
localized lesion from systemic amyloidosis.4,5 In addition immunohistochemical staining or immunofixation electrophoresis of biopsy specimens with specific antibodies may accurately classify this disease. Our patient had renal involvement with ESRD status post– kidney transplantation 6 years prior. He was positive for amyloid on gastric biopsy. The gastrointestinal tract is one of the regions commonly involved in systemic amyloidosis; however, disease confined to the stomach is a rare occurrence.6 The clinical manifestations of amyloidosis are often uncharacteristic, varying with the involved organs. As for gastric amyloidosis, a variety of common gastrointestinal symptoms, such as epigastric discomfort, poor appetite, hematemesis, hematochezia, and gastric perforation may occur in the process of this disease, because of involvement of the local autonomic nervous system and damage to the structure of the gastric wall.7 The patient reported herein complained of dyspeptic symptoms with vomiting; he had gastric involvement of systemic amyloidosis. Erithromycin was administered as on-demand therapy for his complaints, due to its prokinetic properties. No other treatment was deemed necessary, since the patient was symptom-free at 3-month clinical follow-up. In conclusion, although gastric amyloidosis is rare, it should be considered among the possible diagnoses in
Fig 1. 99mTc-labeled meal scintigraphy revealed a T50 of the emptying phase of 194 minutes.
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Fig 2. The control scintigraphy after erythromycin treatment revealed a T50 of the emptying phase of 100 minutes.
patients with dyspeptic complaints and clinical findings of delayed gastric emptying. We suggest that erithromycin treatment may be useful in the supportive therapy of patients with delayed gastric emptying due to systemic secondary amyloidosis. REFERENCES 1. Khan MF, Falk RH: Amyloidosis. Postgrad Med J 77:686, 2001 2. Celik A, Saglam F, Dolek D, et al: Outcome of kidney transplantation for renal amyloidosis: a single-center experience. Transplant Proc 38:435, 2006 3. Berkun Y, Padeh S, Reichman B, et al: A single testing of serum amyloid A levels as a tool for diagnosis and treatment dilemmas in familial Mediterranean fever. Semin Arthritis Rheum 23, 2007 [Epub ahead of print] 4. Bouke P, Hazenberg C, Bijzet J, et al: Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis. Amyloid 14:133, 2007 5. Yoshimatsu SI, Ando Y, Terazaki H, et al: Endoscopic and pathological manifestations of the gastrointestinal tract in familial amyloidotic poyneuropathy type-1 (Met30). J Intern Med 243:65, 1998 6. Wu D, Lou JY, Chen J, et al: A case report of localized gastric amyloidosis. World J Gastroenterol 9:2632, 2003 7. Björnsson S, Johannsson JH, Sigurjonsson F: Localized primary amyloidosis of the stomach presenting with gastric hemmoraghe. Acta Med Scand 221:115, 1987
