Treatment of active steroid-refractory inflammatory bowel diseases with granulocytapheresis： Our experience with a prospective study

AIM: To report our experience with the use of granulo-cytapheresis (GCAP) in 14 patients with active steroid-refractory inflammatory bowel disease (IBD) in order to evaluate its efficacy in achieving remission and maintaining a long lasting symptom-free period. METHODS: The activity of the disease was evaluated by clinical activity index (CAI) and endoscopic index (EI) in ulcerative colitis (UC), while by Crohn's disease activity index (CDAI) in Crohn's disease (CD). The patients were treated using the Adacolumn?system, an adsorption column which selectively binds to granulocytes and monocytes. One session/week of GCAP was performed for 5 wk. Steroids were stopped during apheresis. RESULTS: All the patients completed the five-week course showing no complications. At the end of the last session, 93% of patients showed a clinical remission of the disease that persisted for 6 mo. Nine months after the end of the treatment, 60% of the cases maintained remission, while 23% of the patients were still in clinical remission after 12 mo. CONCLUSION: Even if the number of our patients with steroid-refractory IBDs was not big, we can assert that GCAP is well tolerated and effective, especially in the first six months after the treatment, in a significant percentage of cases. The rate of sustained response drops slightly after 6 mo and significantly after 12 mo, however the absence of severe side effects can be a stimulus for further evaluating new schedules of treatment.     

Clinical analysis of primary small intestinal disease:A report of 309 cases

AIM:To evaluate the major clinical symptom,etiology,anddiagnostic method in patients with primary small intestinaldisease in order to improve the diagnosis.METHODS:A total of 309 cases with primary small intestinaldisease were reviewed,and the major clinical symptoms,etiology,and diagnostic methods were analyzed.RESULTS:The major clinical symptoms included abdominalpain(71%),abdominal mass(14%),vomiting(10%),melaena(10%),and fever(9%).The most common diseasewere malignant tumor(40%),diverticulum(32%)and benigntumor(10%).Duodenal disease was involved in 36% of thepatients with primary small intestinal diseases.The diagnosticrate for primary small intestinal diseases by double-contrastenteroclysis was 85.6%.CONCLUSION:Abdominal pain is the most common clinicalsymptom in patients with primary small intestinal disease.Malignant tumors are the most common diseases.Duodenumwas the most common part involved in small intestine.Double-contrast enteroclysis was still the simplest and themost available examination method in diagnosis of primarysmall intestinal disease.However,more practical diagnosticmethod should be explored to improve the diagnostic accuracy.     

Primary intestinal lymphangiectasia with generalized warts

Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.     

Analysis of clinical manifestations of symptomatic acquired jejunoileal d i verticil la r disease

AIM: To analyze systematically our experience over 22 years with symptomatic acquired diverticular disease of the jejunum and ileum, exploring the clinical manifestations and diagnosis of this rare but life-threatening disease. METHODS: The medical records of patients with surgically confirmed symptomatic jejunoileal diverticular disease were retrospectively reviewed. Data collected included demographic data, laboratory results, clinical course (acute or chronic), preoperative diagnosis, and operative findings. Inclusion criteria were as follows: (1) surgical confirmation of jejunoileal diverticular disease and (2) exclusion of congenital diverticula (e.g. Meckel'sdiverticulum). RESULTS: From January 1982 to July 2004, 28 patients with a total of 29 operations met the study criteria. The male:female ratio was 14:14, and the mean age was 62.6卤3.5 years. The most common manifestation was abdominal pain. In nearly half of the patients, the symptoms were chronic. Two patients died after surgery. Only four cases were correctly diagnosed prior to surgery, three by small bowel series. CONCLUSION: Symptomatic acquired small bowel diverticular disease is difficult to diagnose. It should be considered in older patients with unexplained chronic abdominal symptoms. A small bowel series may be helpful in diagnosing this potentially life-threatening disease.     

New tumor-associated antigen SC6 in pancreatic cancer

AIM: To examine the concentration of a new antigen SC6 (SC6-Ag) recognized by monoclonal antibody (MAb) in patients with pancreatic cancer and other malignant or benign diseases and to understand whether SC6-Ag has any clinical significance in distinguishing pancreatic cancer from other gastrointestinal diseases. METHODS: Six hundred and ninety-five serum specimens obtained from 115 patients with pancreatic cancer, 154 patients with digestive cancer and 95 patients with non-digestive cancer were used and classified in this study. Serum specimens obtained from 140 patients with benign digestive disease and 89 patients with non-benign digestive disease served as controls. Ascites was tapped from 16 pancreatic cancer patients, 19 hepatic cancer patients, 16 colonic cancer patients, 10 gastric cancer and 6 severe necrotic pancreatitis patients. The samples were quantitated by solid-phase radioimmunoassay. The cut-off values (CV) of 41, 80, and 118 U/mL were used. RESULTS: The average intra- and interassay CV detected by immunoradiometric assay of SC6-Ag was 5.4% and 8.7%, respectively. The sensitivity and specificity were 73.0% and 90.9% respectively. The levels in most malignant and benign cases were within the normal upper limit. Among the 16 pancreatic cancer cases, the concentration of SC6-Ag in ascites was over the normal range in 93.8% patients. There was no significant difference in the concentration of SC6-Ag. Decreased expression of SC6-Ag in sera was significantly related to tumor differentiation. The concentration of SC6-Ag was higher in patients before surgery than after surgery. The specificity of SC6-Ag and CA19-9 was significantly higher than that of ultrasound and computer tomography (CT) in pancreatic cancer patients. Higher positive predictive values were indicated in 92.3% SC6-Ag and 88.5% CA19-9, but lower in 73.8% ultrasound and 76.2% CT. CONCLUSION: The combined test of SC6-Ag and CA19-9 may improve the diagnostic rate of primary cancer. The detection of SC6-Ag is valuable in the diagnosis of pancreatic cancer before and after surgery.     

Epidemiological characteristics of inflammatory bowel disease in North-Eastern Poland

AIM: To provide the clinical and epidemiological data of inflammatory bowel disease (IBD) patients of North-Eastern Poland. METHODS: A total of 248 IBD patients diagnosed and hospitalized in the Department of Infectious Diseases in Bialystok between 1990 and 2003 were included in the study. We analyzed age, sex, education, characteristics of job, type of the environment, discontinuation of employment due to IBD, colitis extent, need of surgical treatment, and coexistence of other diseases. RESULTS: Two hundred and thirty-three IBD patients (94%) were diagnosed as ulcerative colitis (DC), and only 15 (6%) were diagnosed as Crohn's disease (CD). Patients with CD were significantly younger at the time of diagnosis and male predominance was observed. The mean age of the patients at the time UC diagnosis was 44.9±1.1 years. Histogram of the age of patients showed the characteristic biphasic distribution with two peaks between 20 and 40 years and between 60 and 70 years. The predominant form of UC was left sided colitis, which affected almost 80% of the studied population. The most extensive form - pancolitis was present in 34 patients (15%). Only 6% of UC patients required surgery, whereas 36% of CD patients underwent surgery (P<0.005). Among coexisting disorders, cholelithiasis was the most prevalent and demonstrated in 35 patients (14%), pulmonary disorders were diagnosed in 2%, and psoriasis in 1.4%. Since 1998, the number of admitted IBD patients has slightly increased. CONCLUSION: Occurrence of UC in Poland is much higher than that of CD. The majority of UC cases are diagnosed in young people (20-40 years) with the predominance of male patients. The most common clinical form of UC is left sided colitis.     

Regulatory T cells in patients with inflammatory bowel diseases treated with adacolumn granulocytapheresis

AIM： To investigate if the clinical efficacy of granulocytes and monocytes by adsorption （GMA） is associated with an increased frequency of peripheral regulatory T cells （Tregs）, as these cells have proven to be successful in suppressing inflammatory bowel disease （IBD） in animal models. METHODS： We report four cases of corticosteroiddependent ulcerative colitis （UC） and two Crohn’s disease （CD） cases with severe cutaneous lesions who received GMA therapy. The frequency of CD4＋ CD25^high （Tregs） in peripheral blood was analyzed by flow cytometry and the expression of FoxP3 and TGF beta in purified CD^4＋ T cells was determined by real time PCR prior to and one month after the last apheresis session, and at the time of endoscopic and clinical assessing. RESULTS： Increased expression of Fox P3 mRNA was found in all five patients who responded to cytapheresis with remission of clinical symptoms, mucosal inflammation and cutaneous lesions, and an increased frequency of circulating Tregs was found in four patients. These changes were not observed in the patient with UC who did no respond to GMA. Variations in TGF-β （mRNA） did not parallel that of FoxP3 mRNA. CONCLUSION： The clinical efficacy of GMA on IBD and related extra intestinal manifestations was associated with an expansion of circulating CD^4＋ CD25^＋ Tregs and higher expression of FoxP3 in CD^4＋ T cells. Accordingly, an elevated CD^4＋ CD25^＋ FoxP3 may be a valuable index of remission in patients with IBD and other chronic relapsing-remitting inflammatory conditions during treatment with GMA.     

Primary small cell carcinoma of esophagus:Report of 9 cases and review of literature

AIM:To analyze the clinical manifestations,pathologicalfeatures and treatment of primary small cell carcinoma (SCC)of the esophagus and to review the literature on this entity.METHODS:The records of 9 patients with primaryesophageal small cell carcinoma were examined and thedemographic data,presenting symptoms,methods of tumordiagnosis,and types of treatment given,response totreatment,pathologic findings,and clinical outcome werereviewed.Features of mixed patterns of histologicaldifferentiation and lymph node metastases were specificallysought.RESULTS:All the patients reported dysphagia,weight lossand chest pain as the initial symptoms.In 5 cases thetumors were located in the mid-esophagus,3 cases in thelower third of the esophagus and 1 case in the upper third.The average length of esophageal involvement was 5 cm.They underwent radical resection,regional lymph nodeclearance and esophageal-stomach anastomosis in thoraxor at neck.Two patients had a stage Ⅱa disease,five hada stage IIb disease,and the other two had a stage Ⅲdisease of International Union Contrele Cancer (UICC).Allof them were histologically and immunohistochemicallyconfirmed SCC of esophagus.Immunohistochemical stainingfor neuron-specific enolase (NSE),synaptophsin (Syn) andchromogranin A exhibited strong immunoreactivity in allspecimens.Three of the nine resected specimens showedfoci of squamous cell carcinoma in situ.Metastasis waspresent in 7 of 9 adjacent lymph nodes.All the patientssurvived the operations and made an uneventful postoperativerecovery.They received adjuvant systemic chemotherapyand local radiation therapy after discharge.During follow-up,three patients developed multiple liver,brain,lung andbone metastases and died between 5 and 18 mo after thediagnosis.Three patients developed widespread metastasisdisease and died between 18 and 37 mo after the diagnosis.There was no local tumor recurrence in these 6 patients.The other three patients were lost during follow-up.CONCLUSION:Primary small cell carcinoma of the esophagusis a rare but very malignant tumor.Radical resectioncombined with chemotherapy and radiotherapy is helpfulin limited stage cases.     

Role of soluble triggering receptor expressed on myeloid cells in inflammatory bowel disease

AIM: To investigate the probable role of soluble triggering receptor expressed on myeloid cells-1 (sTREM-1) in the pathogenesis of inflammatory bowel disease (IBD). METHODS: Fifty-eight patients were enrolled; nineteen healthy volunteers served as controls; 8 patients were diagnosed with Crohn's disease, and 31 with ulcerative colitis. Clinical and endoscopic activity indexes of patients with Crohn's disease and ulcerative colitis respectively were estimated. Upon admission blood was sampled; sTREM-1 and TNFαwere measured by an immunoassay and malondialdehyde (MDA) by the thiobarbitourate assay, after passage through an HPLC system. RESULTS: Median±SE of TNFαof controls, patients with Crohn's disease and patients with ulcerative colitis were 6.02±3.94, 7.98±5.08 (P = NS vs controls), and 8.45±4.15 ng/L (P = 0.018 vs controls) respectively. Respective values of sTREM-1 were 53.31±32.93, 735.10±197.17 (P = 0.008 vs controls) and 435.82±279.71 ng/L (P = 0.049 vs controls). sTREM-1 was positively correlated with Crohn's disease activity index and clinical and endoscopic activity indexes of ulcerative colitis (P = 0.002, 0.001 and 0.009, respectively). sTREM-1 of patients with ulcerative colitis was positively correlated with TNFa (P = 0.001). CONCLUSION: sTREM-1 seems to behave as a novel mediator in IBD in correlation with the degree of the inflammatory reaction of the intestinal mucosa.     

Protein-losing enteropathy in systemic lupus erythematosus: analysis of the clinical features of fifteen patients.

OBJECTIVE: Protein-losing enteropathy (PLE) is an unusual manifestation of systemic lupus erythematosus (SLE), so its clinical manifestations and management are not well understood. In this study, we try to characterize the basic clinical features and the management of PLE by retrospectively analyzing the clinical data of 15 PLE patients and hope this study can improve the awareness of PLE in lupus patients with severe hypoalbuminemia that could not be explained by other causes. METHODS: The clinical data of 15 SLE patients with PLE hospitalized during November 2001 and April 2006 in Peking Union Medical College Hospital were retrospectively reviewed. The PLE was diagnosed by Tc-99m albumin scintigraphy (99mTc-HAS). The clinical characteristics, laboratory tests, response to treatment, and the outcome were studied. RESULTS: The mean age of PLE onset was 40.1 +/- 15.4 years (19-71 years). Twelve were female and 3 were male. 53.3% (8 of 15) patients had PLE as the initial presentation of SLE. All patients had different degree of peripheral pitting edema. Eleven had ascites, 9 had pleural effusion, and 7 had pericardial effusion. Only 6 patients presented with abdominal pain and diarrhea. Positive antinuclear antibodies (HEP-2) with a speckled pattern were found in all patients, but the antidsDNA antibody was negative in most cases. All patients had marked hypoalbuminemia, 80% had hypocomplementemia, 66.7% had hyperlipoproteinemia, and 40% had hypocalcemia. The liver function tests and the prothrombin time were in normal ranges. The 24-hours urine protein was less than 0.5 g in 60% (9 of 15) and more than 1.0 g in 20% (3 of 15) patients who were renal biopsied but only found to have very mild pathologic changes. Gastrointestinal endoscopy examination discovered generalized edema in the intestinal wall whereas the biopsy showed chronic inflammation only. Most cases had good response to corticosteroid and immunosuppressive therapies. The serum albumin level improved evidently in all patients after treatment and normal scintigraphic finding was found in 9 patients. CONCLUSION: PLE can be the initial presentation of SLE or can develop a very long time after the diagnosis of SLE. The prominent clinical presentations are caused by hypoalbuminemia. 99mTc-HAS is useful not only for the diagnosis of PLE but is also helpful for monitoring the efficacy of treatment. When a SLE patient presents with evident hypoalbuminemia without evidence of other causes, PLE should be considered. Early diagnosis and treatment may improve the prognosis.     

蛋白丢失性肠病的临床特点分析

目的概括蛋白丢失性肠病(protein losing enteropathy,PLE)的临床特点及诊治情况。方法以"蛋白丢失性肠病"或"小肠淋巴管扩张"为主题词,在万方、维普、CNKI检索我国2000～2010年公开发表的PLE病例报道。结果纳入的77份病例均除外摄入不足、肝脏合成减少和肾脏丢失,并且证实蛋白从肠道丢失或有影像学和内镜下的诊断依据。其中男30例,女47例,性别比为1∶1.57,成人起病平均年龄(41.06±5.88)岁,儿童起病平均年龄(5.57±2.33)岁。男女起病年龄与病程差异均无统计学意义。本文PLE的病因儿童组以小肠淋巴管扩张症(55.6%)为主,成人组以系统性红斑狼疮(43.9%)为主。PLE的首发症状为浮肿(84.4%),其次分别为腹泻(48.1%)、腹胀(31.2%)、腹痛(28.6%)、消瘦乏力(18.2%)等。结论国内PLE以中年为主,女性多见,起病年龄与病程无性别差异。由于缺乏特异性的临床表现,容易误诊为其他疾病。病程迁延,但预后相对较好。     

A case of systemic lupus erythematosus presenting with intestinal lymphangiectasia-associated protein-losing enteropathy accompanying hyperinflammation

Systemic lupus erythematosus (SLE) has the potential to affect virtually every organ; however, gastrointestinal system manifestations are relatively rare compared to other autoimmune diseases such as systemic sclerosis and inflammatory bowel disease. A 29-year-old female patient attended to the emergency room with abdominal distention, acute onset abdominal pain and constipation. She had watery chronic diarrhea (4-5 times/d) and weight loss (6 kg, 12%) for 4 months. While there was increased intestinal wall thickness, air-liquid levels were shown on abdomen computed tomography scan. The patient underwent abdominal surgery due to diagnosis of ileus. Ileocecal resection was performed and pathologic evaluation revealed intestinal lymphangiectasia. Autoimmune serology was performed with the following resulats: anti-nuclear antibody 1/3200 with homogenous pattern, anti-DNA antibody and anti-Sm/ribonucleoprotein antibodies were positive in addition to low complement levels (C3: 0.28 [0.9-1.8 g/L], C4: 0.06 [0.1-0.4 g/L]) indicating diagnosis of SLE. Development of intestinal involvement in SLE (lupus enteritis) is mainly grouped into 3 headings such as mesenteric vasculitis, pseudo-obstruction, and protein-losing enteropathy. Although the pathogenesis of intestinal lymphangiectasia remains unknown, it has been reported that immune complex-mediated visceral vasculitis may result in bowel wall and mucosal edema. To our knowledge this is the first case report accompanying hyperinflammatory response in addition to intestinal lymphangiectasia in SLE. On the other hand, clinicians should be alert for other reasons for hyperinflammatory syndromes rather than COVID-19, even during the pandemic.     

Lymphangiectasies intestinales primitives (maladie de Waldmann)

Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to lymph leakage into the small-bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main symptom is bilateral lower limb edema. Edema may be moderate to severe including pleural effusion, pericarditis or ascites. Protein-losing enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance and diagnosis by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of biopsies. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Several B-cell lymphomas of the gastrointestinal tract or with extra-intestinal localizations were reported in PIL patients. A long-term strictly low-fat diet associated with medium-chain triglyceride and liposoluble vitamin supplementation is the cornerstone of PIL medical management. Octreotide, a somatostatin analog, have been proposed with an inconsistent efficacy in association with diet. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. A prolonged clinical and biological follow-up is recommended.     

Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)

Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.     

A case of protein-losing enteropathy caused by sclerosing mesenteritis diagnosed with capsule endoscopy and double-balloon endoscopy

A 75-year-old man presented with abdominal distension, hypoproteinemia, ascites and a 35-mm mass in the small bowel mesentery. Laparotomy was performed, and he was diagnosed with sclerosing mesenteritis. His clinical condition improved, with computed tomography (CT) showing tumor shrinkage and decreasing ascites after administration of prednisolone; however, on drug withdrawal, abdominal fullness recurred and CT revealed an enlarging tumor and increasing ascites. Capsule endoscopy (CE) and double-balloon enteroscopy (DBE) were performed to further investigate hypoalbuminemia, which revealed white villi, white nodules, white debris, and mucosal edema in the jejunum. Biopsies from the jejunal mucosa demonstrated infiltration by chronic inflammatory cells consisting mostly of lymphocytes and plasma cells, with marked lymphangiectasia of the lamina propria and submucosa. A fecal alpha-1-antitrypsin clearance test revealed abnormal leakage from the gastrointestinal tract, confirming that hypoalbuminemia was secondary to protein-losing enteropathy (PLE). The incidence of sclerosing mesenteritis accompanied by PLE is very rare. Only six cases have been reported so far. CE and DBE were helpful for diagnosing this condition, and should be performed in patients in whom the cause of hypoalbuminemia is unknown, and in those with PLE.     

Exudative enteropathy in disseminated lupus erythematosus. Report of 3 cases and review of the literature

Protein-losing enteropathy (PLE) is characterized by loss of essentially protein substances into the gastrointestinal tract. Few reports of PLE supervening in patients who have systemic lupus erythematosus (SLE) have appeared in the literature. We report three new cases. All three were women who had a severe form of SLE involving several organs. PLE was diagnosed on the basis of an increased clearance of alpha 1 antitrypsin. The severeness of the clinical picture in all three patients justified the use of immunosuppressive agents (corticosteroids and pulse cyclophosphamide therapy) which were effective. These cases are compared to the 24 previously reported. The frequency of PLE during an SLE flare-up is probably underestimated. It should be looked for in SLE patients who have edema by means of the simple alpha 1 antitrypsin test. PLE is often found in severe clinical forms of SLE and should be managed using corticosteroids either alone or in association with immunosuppressive drugs.     

Protein-losing enteropathy exacerbated with the appearance of symptoms of systemic lupus erythematosus

A 38-year-old woman visited our hospital with edema on her face and conjunctivae. The underlying disease was not clarified, and she did not visit the hospital afterwards. She suffered from diarrhea, polyarthralgia, Raynaud's phenomenon, malar rash and hair loss in the subsequent two years, and was hospitalized because of hypoproteinemia. Her urine, liver and heart test results did not account for her hypoproteinemia. She was diagnosed as having protein-losing enteropathy (PLE) associated with SLE based on the 99mtechnetium-labeled human serum albumin scintigraphy findings, clinical findings and laboratory results of antinuclear and anti-Sm antibodies. This case report demonstrates a strong association between PLE and SLE because PLE was aggravated along with the appearance of SLE symptoms and PLE subsided with prednisolone treatment along with improvement of SLE.     

Successful treatment of protein-losing enteropathy due to AA amyloidosis with octreotide in a patient with rheumatoid arthritis

Protein-losing enteropathy (PLE) is a rare syndrome of gastrointestinal protein loss that may complicate a variety of diseases. This excessive protein loss across the gut epithelium can be explained by several mechanisms, such as augmentation of the intestinal mucosal capillary permeability, mucosal disruption, intestinal or mesenteric vasculitis, and lymphangiectasia. However, these pathophysiologic alterations of the gut are closely linked to the underlying cause, and primary treatment for PLE should be directed at the underlying condition. Here, we report a female patient with rheumatoid arthritis who developed severe PLE due to AA amyloidosis and was successfully treated with octreotide. She had been suffered from rheumatoid arthritis for 18 years, and her arthritic symptoms at the time of presentation were not definite but manifested as severe diarrhea and general edema with hypoalbuminemia. PLE due to gastrointestinal amyloidosis was confirmed by increased fecal α₁-antitrypsin clearance and a colonoscopic biopsy that was positive for amyloid deposits. The diarrhea dissipated with conventional treatment, but the general edema resolved only after introducing a long-acting somatostatin analog (octreotide), along with a gradual recovery of the serum albumin level. This case teaches us that in the case of PLE due to AA amyloidosis that is refractory to conventional treatment, the administration of octreotide should be considered.