A Case with Multiple Colonic Ulcers Simulating Churg-Strauss Syndrome

Abstract: Churg-Strauss syndrome (CSS), a relatively rare disorder which is associated with serious complications, has a highly variable course and several possible manifestations. We present the case of a 35-year-old woman with a history of bronchial asthma, admitted for evaluation of lower abdominal pain and melena, whose clinical course had certain features in common with CSS. On admission, the white blood cell count was 45,300/mm³ with 65% eosinophils, and the serum immunoglobulin E (5,300 u/ml) level was remarkably elevated. At colonoscopy, there were shallow ulcers, irregular in shape, throughout the large intestine. Abdominal pain and melena were relieved by oral administration of prednisolone. Most previously reported cases have not been recognized as having colonic involvement until surgery or autopsy. In only a few reports of CSS and related disorders were colonoscopic examination findings described.     

Churg–strauss syndrome: importance of colonoscopy and endoscopic biopsy in establishing diagnosis

A 31‐year‐old Japanese man was admitted with complaints of left lower abdominal pain and diarrhea. He had a history of bronchial asthma since he was 26 years old. On admission, his eosinophil count was 4100/mm ³ and increased as high as 12 000/mm ³ . A neurological examination disclosed mononeuritis multiplex with paresthesia in both lower and upper extremities. Because of hematochezia, colonoscopy was performed and disclosed multiple colonic ulcers and erosions from the rectum to the ascending colon. Endoscopic biopsy showed fibrinoid necrosis of small‐sized vessels with moderate infiltration of eosinophils in the mucosa and submucosa. He was suspected of being a case with Churg–Strauss syndrome (CSS; allergic granulomatous angiitis), and prednisolone of 30 mg/day was administered. After the administration of prednisolone, his eosinophil count decreased dramatically. His symptoms also improved gradually, except for numbness in the extremities. Three weeks later, colonoscopy revealed improvement of the mucosal appearance, but the colonic ulcers and erosions were still present. Endoscopic biopsy showed exravascular granuloma with moderate infiltration of eosinophils in the submucosa. Colonoscopy and endoscopic biopsy may be important in establishing the diagnosis of CSS with intestinal symptoms.     

ASCARIASIS AS A CAUSE OF RECURRENT ABDOMINAL PAIN

Ascariasis is the most common helminthic infection in developing countries. It may cause chronic abdominal pain, tenderness and bloating. Our aim is to report a case of acute episodic abdominal pain and pancreatitis associated with ascariasis. We report a 59‐year‐old female patient who was admitted for acute abdominal pain, having had several previous similar events before one of them was diagnosed as acute idiopathic pancreatitis. On admission, her physical exam was normal. Laboratory results showed hemoglobin 12.2 g/dL, white blood cell count 11 900 cells/mm³, eosinophils 420 cells/mm³, serum amylase 84 IU/mL, lipase 22 IU/mL and normal liver function tests. Abdominal ultrasound and a plain abdominal X‐ray were also normal. An upper endoscopy showed round white worms in the duodenum and the stomach, some of them with bile in their intestines. The intestinal parasites were diagnosed as Ascaris lumbricoides, and the patient was started on albendazole, with full recovery within a week. We believe that ascariasis should be considered in patients with recurrent abdominal pain and idiopathic pancreatitis.     

Cases from the Osler Medical Service at Johns Hopkins University

Presenting features

A 41-year-old man with a past history of alcohol abuse, drug abuse, and smoking (>40 pack-years) presented with chronic epigastric pain and weight loss. During the preceding year, the epigastric pain had worsened progressively and was exacerbated by eating. For the past 3 months, his nausea and vomiting had worsened so that he was no longer able to tolerate solid food. Consequently, he had lost 50 lbs in 1 year. During the 3 weeks before admission, he had daily subjective fevers but no night sweats. He denied any melena, hematemesis, jaundice, or changes in stool color or consistency.His past medical history was remarkable for an episode of massive hematemesis, for which he was admitted to another hospital 3 months ago and found to have a bleeding gastric ulcer. Despite treatment with a proton pump inhibitor, his abdominal pain, nausea, and vomiting worsened after dis-charge. He stopped drinking alcohol after that hospitalization, and he stopped using intravenous drugs 1 month before this admission.On physical examination, the patient appeared wasted. His vital signs were notable for a normal blood pressure with resting tachycardia and an orthostatic increase in pulse rate. His jugular veins were flat. Cardiac and respiratory examinations were normal. The abdominal examination revealed guarding in the epigastric region with hypoactive bowel sounds and no rebound tenderness. His stool was guaiac negative. Laboratory studies revealed a total white blood cell count of 4180/mm³, a hematocrit of 33.1%, a platelet count of 239,000/mm³, a total protein level of 8.3 g/dL, an albumin level of 3.7 g/dL, and antibodies to human immunodeficiency virus (HIV). A computed tomographic scan of the abdomen showed thickening of the antrum with large gastric folds.What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University. Zieve syndrome

Presenting features

A 47-year-old African American man was admitted to the Osler Medical Service with a chief complaint of light-headedness. He was a heavy drinker and consumed 2 pints of fortified wine every day. On the day of admission, he had been at home consuming alcohol when he stood up, became lightheaded, and immediately had to sit down. He denied any loss of consciousness, dyspnea, hematemesis, coffee-ground emesis, lower abdominal pain, bright red blood per rectum, or melena, but he recalled a burning epigastric discomfort. The patient's medical history was notable only for a 20-year history of hypertension and recently diagnosed type 2 diabetes mellitus. He was not taking any medications. He had a normal hematocrit of 43% 6 months prior to admission.Physical examination showed a supine heart rate of 115 beats per minute and blood pressure of 165/90 mm Hg, without orthostatic changes. There was scleral icterus and a jaundiced palate, but no other stigmata of end-stage liver disease. His abdominal examination was unremarkable; there were no masses, tenderness, or hepatosplenomegaly. Rectal examination revealed guaiac-negative stool. The chest radiograph and electrocardiogram were unremarkable.On admission, the laboratory examination was notable for the following values: hematocrit, 22.8% with a mean corpuscular volume of 86.7 fL and a red cell distribution width of 25.7%; absolute reticulocyte count, 177,500/mm³; total bilirubin, 4.2 mg/dL; direct bilirubin, 2.3 mg/dL; albumin, 3.3 g/dL; lactate dehydrogenase, 481 U/L; aspartate aminotransferase, 106 U/L; and alanine aminotransferase, 44 U/L. The prothrombin and activated partial prothrombin times were normal. There was no evidence of iron, vitamin B₁₂, or folate deficiency. The patient's haptoglobin level was severely depressed (<6 mg/dL) with a negative direct Coomb test and normal glucose-6-phosphate dehydrogenase activity. A peripheral blood smear was compatible with hemolysis and demonstrated normocytic, normochromic erythrocytes with moderate poikilocytosis, as well as rare spherocytes and target cells Figure 1).On the second day of hospitalization, a lipid panel revealed hypercholesterolemia with a total cholesterol level of 300 mg/dL. Due to the patient's complaint of burning epigastric pain and long history of alcohol consumption, esophagogastroduodenoscopy was performed and revealed grade 1 nonbleeding esophageal varices.What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University. Hodgkin's disease with Pel-Ebstein fevers

Presenting features

A 58-year-old man presented to the Johns Hopkins Hospital complaining of a 9-month history of malaise, fevers, night sweats, and weight loss. His fevers were episodic in nature with periods of constant fevers reaching high peaks (104°F) during the day from 5 to 7 days followed by an afebrile period 3 to 4 weeks. A typical fever curve of these episodes is shown in Figure 1 Note that the patient’s temperature rarely drops below the febrile range, <38°C. Review of symptoms was noteworthy for the lack of headache, pharyngitis, sinus congestion, cough, abdominal pain, diarrhea, arthritis, rashes, dysuria, urinary frequency, or neurologic symptoms. He had been placed on numerous courses of antibiotics, including levofloxacin and clarithromycin, during the months before ad-mission without a change in his symp-toms. Past medical history was unremarkable. He was on no medications. He denied recent travel, exposure to farm or exotic animals, or illicit drug use. He had been monogamous with his wife.His admission physical examination was notable for a temperature of 104.4°F and firm enlarged (1.5 to 2 cm) cervical and axillary lymph nodes. Admission laboratory data were notable for elevated liver function enzyme (aspartate aminotransferase 50, alanine aminotransferase 73, alanine phosphatase 274), and pancytopenia with a white blood cell count of 1300/mm³ (but a normal white cell differential), hematocrit level of 26, and platelet count of 103.000/mm³. To elucidate the etiology of his febrile episodes, a diagnostic procedure was performed.What is the diagnosis?     

A case of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura

A 30-year-old Japanese woman with chest pain and nodules in the left upper lung field was diagnosed as having pulmonary tuberculosis by sputum examination. Purpura on her legs had lasted for 3 months and her platelet count was 1.9 × 10⁴/mm³ on admission. She was also diagnosed as having immune thrombocytopenic purpura because of elevation of serum PA-IgG and proliferation of megakaryocytes in the bone marrow. Anti-tubercular therapy and steroid therapy were concurrently performed, resulted in recovery of the platelet count. Steroid therapy was gradually tapered off and then withdrawn, thereafter anti-tubercular therapy was finished. She has been relapse-free.Cases of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura are rare. The pathogenesis in the present case was suggested to have occurred through an immunological mechanism.     

Leukocyte count in the synovial fluid of children with culture-proven brucellar arthritis

Brucellosis is an important cause of paediatric septic arthritis in endemic areas. Because the Gram stain is frequently negative and culture results are unavailable at the time of the patient’s admission, the diagnosis of brucellar arthritis is usually entertained on the bases of epidemiological considerations and cytological examination of the synovial fluid aspirate. The aim of this study was to assess the sensitivity of a synovial fluid leukocyte count >50 000 WBC/mm³ for detecting culture-proven brucellar arthritis in children. The medical records of all children with brucellar arthritis diagnosed since 1994 in a hospital serving an endemic area for brucellosis in southern Israel were reviewed. Nine patients (six males and three females), aged 3–14 years, were identified. A single joint was affected in all patients. The median leukocyte count in the synovial fluid was 9500 WBC/mm³ (range 300–61 500 WBC/mm³), and in eight of the nine patients it was less than 50 000 WBC/mm³. Brucella melitensis was recovered from the synovial fluid culture in all patients. The diagnosis of brucellar septic arthritis cannot be excluded on the basis of a low leukocyte count in the joint aspirate. A high index of suspicion and use of modern culture techniques are recommended to improve the diagnosis of brucellar arthritis. Received: 24 March 2001 / Accepted: 29 October 2001     

A case of fulminant amoebic colitis with an abscess in the abdominal cavity rescued by conservative management

A 75-year-old man was admitted because of watery diarrhea, hematochezia and right lower abdominal pain. Many deep undermining colonic ulcers were found by colonoscopy, and we detected trophozoite amoeba pathologically. Metronidazole was administered orally from 3 days after admission. However, since CT demonstrated a huge abscess in the abdominal cavity, we performed percutaneous drainage from 17 days after admission. On day 157, the patient was discharged, because the colonic ulcers had almost healed, and trophozoite amoebas were not recognized pathologically.     

Corticosteroid-resistant multiple colonic ulcers in a patient with systemic lupus erythematosus/systemic sclerosis overlap syndrome effectively treated with intravenous cyclophosphamide pulse therapy

We report a rare case of a 17-year-old female with overlap syndrome (systemic lupus erythematosus and systemic sclerosis) who developed severe abdominal pain and bloody diarrhea accompanied by central nervous system lupus. Colonoscopy revealed multiple irregular and linear ulcers throughout the colon, which were resistant to corticosteroid pulse therapy and plasma exchange. The patient finally recovered after treatment with a relatively low dose of monthly intravenous cyclophosphamide (250mg/m²) pulse therapy.     

Acute appendicitis following endoscopic mucosal resection of cecal adenoma

Endoscopic mucosal resection (EMR) allows the removal of flat or sessile lesions, laterally spreading tumors, and carcinoma of the colon or the rectum limited to the mucosa or the superficial submucosa. Acute appendicitis is the most common abdominal emergency requiring emergency surgery, and it is also a rare complication of diagnostic colonoscopy and therapeutic endoscopy, including EMR. In the case presented here, a 53-year-old female underwent colonoscopy due to a positive fecal occult blood test and was diagnosed with cecal adenoma. She was referred to our hospital and admitted for treatment. The patient had no other symptoms. EMR was performed, and 7 h after the surgery, the patient experienced right -lower abdominal pain. Laboratory tests performed the following day revealed a WBC count of 16000/mm³, a neutrophil count of 14144/mm³, and a C-reactive protein level of 2.20 mg/dL, indicating an inflammatory response. Computed tomography also revealed appendiceal wall thickening and swelling, so acute appendicitis following EMR was diagnosed. Antibiotics were initiated leading to total resolution of the symptoms, and the patient was discharged on the sixth post-operative day. Pathological analysis revealed a high-grade cecal tubular adenoma. Such acute appendicitis following EMR is extremely rare, and EMR of the cecum may be a rare cause of acute appendicitis.     

Effect of alternative antibiotics in treatment of cefotaxime resistant spontaneous bacterial peritonitis

AIM:To evaluate effective alternative antibiotics in treatment of cefotaxime-resistant spontaneous bacterial peritonitis.METHODS:One hundred cirrhotic patients with spontaneous bacterial peritonitis [ascitic fluid polymorphonuclear cell count(PMNLs) ≥ 250 cells/mm 3 at admission] were empirically treated with cefotaxime sodium 2 g/12 h and volume expansion by intravenous human albumin.All patients were subjected to history taking,complete examination,laboratory tests(including a complete blood cell count,prothrombin time,biochemical tests of liver and kidney function,and fresh urine sediment),chest X-ray,a diagnostic abdominal paracentesis,and the sample subjected to total and differential cell count,chemical examination,aerobic and anaerobic cultures.Patients were divided after 2 d by a second ascitic PMNL count into group Ⅰ;patients sensitive to cefotaxime(n = 81),group Ⅱ(n = 19);cases resistant to cefotaxime(less than 25% decrease in ascitic PMNL count).Patients of group Ⅱ were randomly assigned into meropenem(n = 11) or levofloxacin(n = 8) subgroups.All patients performed an end of treatment ascitic PMNL count.Patients were considered improved when:PMNLs decreased to < 250 cells/mm 3,no growth in previously positive culture cases,and improved clinical manifestations with at least 5 d of antibiotic therapy.RESULTS:Age,sex,and Child classes showed no significant difference between group Ⅰ and group Ⅱ.Fever and abdominal pain were the most frequent manifestations and were reported in 82.7% and 80.2% of patients in group Ⅰ and in 94.7% and 84.2% of patients in group Ⅱ,respectively.Patients in group Ⅱ had a more severe ascitic inflammatory response than group Ⅰ and this was demonstrated by more ascitic lactate dehydrogenase(LDH) [median:540 IU/L(range:150-1200 IU/L) vs median:240 IU/L(range:180-500 IU/L),P = 0.000] and PMNL [median:15 000 cell/mm 3(range:957-23 822 cell/mm 3) vs 3400 cell/mm 3(range:695-26 400 cell/mm 3),P = 0.000] counts.Ascitic fluid culture was positive in 32% of cases.C     

Double-balloon endoscopy-diagnosed multiple small intestinal ulcers in a Churg-Strauss syndrome patient

Churg-Strauss syndrome(CSS) is a systemic vascular disorder characterized by severe bronchial asthma hypereosinophilia,and allergic rhinitis.Small intestina ulcers associated with CSS are a relatively rare manifestation that causes gastrointestinal bleeding.Multiple deep ulcers with an irregular shape are characteristic of small intestinal involvement of CSS.Video-capsuleendoscopy(VCE),double-balloon endoscopy(DBE) and Spirus assisted enteroscopy have been developed recently and enabled observation of the small intestine In this case report,we have described a patient with CSS who had multiple deep ulcers in the jejunum detected by oral DBE.Since severe gastrointestinal(GI) involvement has been identified as an independent factor associated with poor outcome,the careful investigation of GI tract must be needed for CSS patients with GI symptoms.We describe the usefulness of DBE for diagnosis of small intestinal ulcers in patient with CSS.     

Cases from the Medical Grand Rounds of the Osler Medical Service at Johns Hopkins University

Presenting features

A 39-year-old male with acquired immunodeficiency syndrome (CD4 count of 3) presented with the chief complaints of left-sided abdominal pain, fevers, and night sweats of three-weeks duration. He was noncompliant with prophylaxis against Pneumocystis carinii pneumonia and Mycobacterium avium complex and was not currently on highly-active antiretroviral therapy (HAART). Physical examination on admission was noteworthy for a temperature of 39° C and abdominal pain to deep palpation in the left mid-epigastric region. An extensive work-up was undertaken, including an esophagogastroduodenoscopy with enteroscopy which raised papular lesions in his jejunum (Figure 1, arrows). What is the diagnosis?     

Non-surgical contraindication for acute appendicitis with secondary thrombocytopenia: A case report

A 26-year-old man presented with migrated right lower abdominal pain and without any history of hematological systemic diseases.Blood routine test showed a leukocyte count of 22.74 × 109/L, with91.4% neutrophils, and a platelet count of 4 × 109/L before admission.The case question was whether the team should proceed with surgery.Obviously, a differential diagnosis is essential before making such a decision.Acute appendicitis was easily diagnosed based on clinical findings, including migrating abdominal pain, a leukocyte count of 22.74 × 109/L and the result of abdominal computed tomography scan.However, it was not clear whether the severe thrombocytopenia was primary or secondary.So smear of peripheral blood and aspiration of bone marrow were ordered to exclude hematological diseases.Neither of the tests indicated obvious pathological hematological changes.There was no hepatosplenomegaly found by ultrasound examination of the liver and spleen.Therefore, operative intervention may be a unique clinical scenario in acute severe appendicitis patients with secondary thrombocytopenia.     

Intérêt de l’imagerie par résonance magnétique nucléaire au cours de l’atteinte cardiaque du syndrome de Churg-Strauss. Trois observations et revue de la littérature

Purpose

The reported prevalence of cardiac complications is variable in patients with Churg-Strauss syndrome (15–92%) and depends on diagnostic tools. Diagnosis at early stage of heart involvement is crucial, resulting in appropriate management.

Methods

We report three patients who developed cardiac manifestations, revealing Churg-Strauss syndrome. The diagnosis of cardiac involvement was obtained using cardiac magnetic resonance imaging (MRI).

Results

Two patients were males and the remaining one was a female. Presenting clinical manifestations were: cardiac failure (n = 1) and retrosternal pain (n = 2). Laboratory findings disclosed: high blood count of eosinophils (range: 6000–11 000/mm³); antineutrophil cytoplasmic antibodies were positive in a single patient. Cardiac MRI demonstrated: (1) late gadolinium enhancement (n = 3), involving mainly the apical and mid-cavity left ventricular segments; (2) impaired left ventricular function (n = 2), mean left ventricular ejection fraction being: 51%; and (3) pericardial effusion (n = 3). Outcome was favourable after institution of combined therapy with prednisone and cyclophosphamide (n = 2); one patient also underwent plasma exchanges.

Conclusion

Our case series underlines that MRI is a helpful tool in the diagnosis of Churg-Strauss syndrome-related cardiac complications. We further suggest that clinical assessment of patients with Churg-Strauss syndrome should include cardiac MRI, in order to detect cardiac involvement at an early stage; indeed, because cardiac manifestations are predictive factors of poor prognosis, diagnosis at early stages of cardiac involvement may result in improvement of patients management.     

Analysis of clinical course and prognosis of culture-positive spontaneous bacterial peritonitis and neutrocytic ascites

The clinical significance and prognosis of culture-negative neutrocytic ascites in cirrhotic patients is a controversial topic. In the present study, the clinical and humoral presentation and the short-and long-term prognosis were analyzed in 36 patients with cirrhosis and culture-positive spontaneous bacterial peritonitis and in 28 patients with cirrhosis and ascitic fluid polymorphonuclear count greater than 250/mm³, a negative ascitic fluid culture, and without previous antibiotic therapy. On admission there were no significant differences between groups related to age, sex, alcoholism, fever, abdominal pain, serum albumin, serum urea, serum creatinine, Child-Pugh score, polymorphonuclear count, and total protein concentration in ascitic fluid. A greater frequency of positive blood culture was found in patients with spontaneous bacterial peritonitis (15/21 vs 2/18) (P<0.001). Mortality during the first episode was 36% in patients with spontaneous bacterial peritonitis and 46% in patients with culture-negative neutrocytic ascites (NS). Mortality during follow-up was high and survival probability at 12 months was 32% in spontaneous bacterial peritonitis and 31% in culture-negative neutrocytic ascites. The probability of recurrence at 12 months was 33% in spontaneous bacterial peritonitis and 34% in culture-negative neutrocytic ascites. Our results show that spontaneous bacterial peritonitis and culture-negative neutrocytic ascites are variants of the same disease with a high mortality and poor prognosis.     

Recovery from idiopathic thrombocytopenic purpura (ITP) following right hemicolectomy for mucocele of appendix

A 61-year-old woman with a 2-year medication-free history of idiopathic thrombocytopenic purpura was referred to our hospital complaining of right lower abdominal pain. The platelet count was about 3–4 × 10⁴ /mm³. This patient was diagnosed with mucocele of the appendix on computed tomography and colonoscopy. We performed only right hemicolectomy without splenectomy. The pathological diagnosis was mucinous cystadenoma of the appendix. The platelet count increased to 18.1 × 10⁴ /mm³ on postoperative-day 7, and remained stable, at 20–24 × 10⁴ /mm³, for 14 months after the operation. Platelet-associated immunoglobulin G decreased remarkably after the operation, to 30.2 ng/10⁷ cells, from 240 ng/10⁷ cells preoperatively. We describe the first case of recovery from idiopathic thrombocytopenic purpura following right hemicolectomy performed for mucocele of the appendix. Considering our patients clinical course, it is possible that mucinous cystadenoma of the appendix may have influenced the thrombocytopenia. Findings in this patient suggest that mucocele of the appendix may be associated with a new diagnosis and idiopathic thrombocytopenic purpura treatment.     

Corticosteroid-resistant multiple colonic ulcers in a patient with systemic lupus erythematosus/systemic sclerosis overlap syndrome effectively treated with intravenous cyclophosphamide pulse therapy

Abstract

We report a rare case of a 17-year-old female with overlap syndrome (systemic lupus erythematosus and systemic sclerosis) who developed severe abdominal pain and bloody diarrhea accompanied by central nervous system lupus. Colonoscopy revealed multiple irregular and linear ulcers throughout the colon, which were resistant to corticosteroid pulse therapy and plasma exchange. The patient finally recovered after treatment with a relatively low dose of monthly intravenous cyclophosphamide (250?mg/m2) pulse therapy.     

Churg-Strauss syndrome manifesting as cholestasis and diagnosed by liver biopsy

A 56-year-old woman was referred to our hospital due to fever and cholestatic liver dysfunction. Her eosinophil count was normal and she had no abdominal pain or neurological manifestations. We performed a liver biopsy and found fibrinoid necrosis of the hepatic artery with granulomatous reaction and eosinophilic infiltration in the portal area in the liver. Later, sensory abnormalities of the arms and legs appeared and the eosinophil count increased. Serum immunoglobulin E and immunoglobulin G4 were elevated and rheumatoid factor was strongly positive. Endoscopic retrograde cholangiopancreatography revealed no abnormality of the bile duct and pancreatic duct. We made a diagnosis of Churg-Strauss syndrome and began corticosteroid treatment. Fever and liver function immediately improved. In the present patient, Churg-Strauss syndrome manifested first in the liver, before hypereosinophilia and neural manifestations. We believe that Churg-Strauss syndrome is an autoimmune liver disease, and it is important to recognize that the liver may be involved in Churg-Strauss syndrome.