Rare manifestations of Churg–Strauss syndrome: coronary artery vasospasm, temporal artery vasculitis, and reversible monocular blindness—a case report

Churg–Strauss syndrome (CSS) is a rare illness with clinical findings characterized by asthma, eosinophilia, and vasculitis affecting medium and small-sized arteries and veins in a variety of organs. Involvement of the temporal arteries by non-giant cell eosinophilic vasculitis in CSS is quite rare and has only been published as isolated case reports or small patient series. Myocardial infarction due to coronary artery vasospasm is an unusual manifestation of CSS. We describe a case of a 39-year-old woman who had two myocardial infarctions due to severe coronary artery vasospasm and was diagnosed with CSS based on a temporal artery biopsy. During the course of her treatment, she also had another rare manifestation of CSS, monocular blindness reversible with immunosuppressive therapy.     

Tertiary syphilis presenting as hepatic bull's eye lesions

We report a rare case of tertiary syphilis (hepatic gummata, asymptomatic neurosyphilis and iridocyclitis) in a 47 year old female patient. Our patient suffered from a troubled sight, pain in the right hypochondrium, one enlarged submandibular lymph node, an elevated sedimentation rate, disturbed liver tests and two hepatic lesions upon abdominal computed tomography. The diagnosis was based upon a liver biopsy and a positive Treponema Pallidum haemagglutination test. The patient was treated with doxycyclin. After treatment the sedimentation rate and liver tests normalised and the hepatic lesions disappeared leaving a small 'scar' on CT-scan; at the end she still complained of a decreased sight. We conclude that syphilitic gummata of the liver have a favourable prognosis when the diagnosis is made early. One has to differentiate with hepatic abscesses, primary tumours and metastases.     

Ruptured Klebsiella pneumoniae liver abscess after high-dose cyclophosphamide for severe aplastic anemia

A 19-year-old woman with severe aplastic anemia who had previously failed antithymocyte globulin/cyclosporine A received high-dose cyclophosphamide without bone marrow rescue. On day +14, she complained of right upper quadrant abdominal pain and fever. A CT scan of the abdomen showed multiple liver abscesses with rupture and Klebsiella pneumoniae was isolated from blood. In spite of aggressive antibiotic therapy, she rapidly deteriorated and died of overwhelming sepsis. To our knowledge, our patient is the first case of fatal ruptured liver abscess after high-dose cyclophosphamide in a patient with severe aplastic anemia.     

Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome： Case report and review of the literature

INTRODUCTION Gallbladder disease is no more common in patients with systemic lupus erythematosus (SLE) than in the general population[1]. Scarce reports on acalculous cholecystitis (ACC) in SLE or in antiphospholipid syndrome (APS) have been described in …     

Case of emphysematous cholecystitis in a patient with type 2 diabetes mellitus associated with schizophrenia

Emphysematous cholecystitis is a rare, but life‐threatening, form of acute cholecystitis caused by gas‐forming organisms in the gallbladder. A 73‐year‐old male patient with type 2 diabetes mellitus complicated with neuropathy associated with schizophrenia was admitted to Okayama University Hospital, Okayama, Japan, because of a high fever and general malaise. On the fourth hospital day, despite normal liver function tests and little abdominal pain, his abdominal computed tomography showed huge gas formation in the gallbladder lumen along with a dilated gallbladder with a thickened wall, consistent with emphysematous cholecystitis. The patient underwent an emergency open cholecystectomy. Few abdominal symptoms appeared because of the hyposensitivity to pain caused by not only diabetic neuropathy, but also antipsychotic agents the patient was taking for schizophrenia. Emphysematous cholecystitis should be taken into consideration for the differential diagnosis of high fever in diabetic patients with schizophrenia, irrespective of the level of liver function tests and clinical symptoms.     

Rapidly progressive irradiated cervical cancer that metastasized to the liver during therapy for idiopathic thrombocytopenic purpura

A 37-year-old woman was given a diagnosis of cervical cancer in August 1994. Because of severe thrombocytopenia, she was given radiation therapy at 50 Gy with great effectiveness. The thrombocytopenia was diagnosed as idiopathic thrombocytopenic purpura. Because the patient refused to undergo a splenectomy operation, she was treated with prednisolone, gamma-globulin, and danazol with no effect. In January 1995 she began receiving azathioprine and her platelet count gradually increased. In March, she complained of severe left abdominal pain but abdominal computed tomography (CT) scans showed no abnormal findings. Nonetheless, the patient's lumbago persisted and her liver dysfunction was progressive. Abdominal CT scans performed on April 18 disclosed multiple liver tumors. The patient died on April 28. Autopsy revealed that the cervical cancer was the primary origin of the liver tumors. We concluded that extra precautions should be taken when administering immunosuppressive therapy to patients with a history of malignant diseases.     

Idiopathic hypereosinophilic syndrome presenting with liver mass: Report of two cases: Idiopathic hypereosinophilic syndrome and liver mass

Herein, we report on two cases of hypereosinophilic syndrome presenting as liver mass. One patient was a 22-year-old woman presented with fever, upper abdominal pain, nausea/vomiting and a mass in the right liver lobe. The second patient was a 54-year-old man who presented with nausea and abdominal pain with significant weight loss. He had multiple lesions in both liver lobes. Both patients had eosinophilia that was not attributed to other causes such as allergy or parasites. The patients were treated with glucocorticosteroid and improved clinically. Imaging and laboratory abnormalities resolved.     

Patient with perforation caused by emphysematous cholecystitis who showed flare on the skin of the right dorsal lumbar region and intraperitoneal free gas

We report an 84-year-old man with perforation caused by emphysematous cholecystitis who showed flare on the skin of the right dorsal lumbar region and intraperitoneal free gas. The patient was admitted for abdominal pain, abdominal swelling, and consciousness disorder 18 days after the onset. Abdominal computed tomography (CT) revealed emphysema in the gallbladder and a small amount of intraperitoneal free gas. Intraoperative findings suggested gangrenous cholecystitis. The gallbladder wall was perforated, and an abscess involving the right subphrenic region, the periphery of the liver and gallbladder, and the right paracolonic groove, was detected. The flare on the body surface may have reflected abscess formation in the right abdominal cavity. Emphysematous cholecystitis induces necrosis and perforation in many patients, and immediate strategies such as emergency surgery are important.     

2 Imaging and intervention in patients with acute right upper quadrant disease

Because of the high diagnostic yield, its widespread availability and the possibility of bedside examinations, US has become the imaging modality of choice in patients with acute right upper quadrant pain caused by inflammatory disorders such as liver abscesses, acute cholangitis and acute cholecystitis. Computed tomography (CT) can be reserved for more complex cases. US, often in combination with fluoroscopy, is also widely used to control interventions. In patients with liver abscesses the therapeutic strategy is determined by the size of the abscess, its uni- or multifocal presentation and the causative micro-organisms cultured after diagnostic percutaneous aspiration. Small-sized pyogenic abscesses (<3 cm), most fungal and amoebic abscesses can be treated medically. Large-sized pyogenic abscesses should be drained percutaneously and can be cured in 75–90%. Surgery should be restricted to patients with prolonged sepsis after percutaneous drainage and patients with infected pre-existing hepatic lesions.In patients with acute cholangitis drainage of the infected bile is essential. Invasive imaging such as percutaneous or endoscopic cholangiography should only be done with the intention to drain. The use of endoscopic procedures such as nasobiliary drainage, stent placement and sphincterotomy has decreased mortality rates dramatically. Percutaneous drainage should be considered in patients in whom endoscopic procedures fail. Surgery may have a place in the treatment of bile duct obstruction which causes cholangitis.In patients with suspected acute cholecystitis, imaging modalities such as cholescintigraphy and CT can be reserved for patients with inconclusive sonographic studies and more complex cases. The contribution of percutaneous gallbladder aspiration and culture to diagnose acute cholecystitis seems limited. Percutaneous cholecystostomy is an effective procedure with a low morbidity and mortality for high-risk patients. The drainage catheter in the gallbladder does not interfere with cholecystectomy at a later stage in patients with calculous cholecystitis. In most patients with acalculous cholecystitis, percutaneous cholecystectomy provides a definitive treatment.     

Acute cholecystitis and duodenitis associated with Churg-Strauss syndrome

We describe a patient with acute cholecystitis and duodenitis associated with Churg-Strauss syndrome. A 36-year-old male, who had been healthy, had abdominal pain following high fever. He had marked hypereosinophilia of 17,000/mm3. Radiographs of the chest disclosed a transient infiltrated lesion in the left lower lung. Ultrasonographic and gastroendoscopic examinations revealed acute cholecystitis and duodenitis, respectively. Endoscopic retrograde cholangiopancreatography demonstrated a filling defect suspecting aberrant ascariasis in the common bile duct. The patient suddenly developed distally dominant mononeuritis multiplex, especially in the upper limbs. Muscle biopsy revealed vasculitis of intramuscular arteries with infiltration of eosinophils. These findings fulfilled the diagnostic criteria of Churg-Strauss syndrome. Corticosteroid dramatically resolved the abdominal symptoms. Cholecystectomy and removal of the foreign body were performed. Histological examinations revealed that necrosis of the gallbladder was caused by occlusion due to thrombosed arteries and that the foreign body in the common bile duct was an aggregate of necrotic epithelium of the bile duct wall surrounded by inflammatory cells. Although abdominal complaints rarely appeared as an initial symptom in the patients with Churg-Strauss syndrome, this syndrome should be taken into consideration for an accurate diagnosis when the patients with abdominal pain of unknown origin had eosinophilia, asthma, or allergic rhinitis.     

Fasciola hepatica infection: clinical and computerized tomographic findings of ten patients.

BACKGROUND/AIMS: Fasciola hepatica is the cause of liver infection, fascioliasis. Although rare, it is still a problem even in developed countries. In this study, the clinical and computerized tomographic findings of 10 patients diagnosed with fascioliasis are summarized. METHODS: The medical records of the patients with fascioliasis were retrospectively examined. Clinical, laboratory findings and computerized tomographic results were recorded. RESULTS: Abdominal pain, fever, eosinophilia and abnormal liver function tests were the most commonly encountered symptoms and signs. One patient was human immunodeficiency virus -positive with active tuberculosis. Serologic test for fasciola hepatica was positive in all patients. Nodular masses without prominent enhancement, and branching low-attenuated tubular lesions were the most commonly seen tomographic findings and were supportive for the diagnosis. All except the HIV-positive patient received bithionol therapy; six patients responded well, two lost contact with the clinic and one patient who was unresponsive to bithionol therapy received triclabendazole. During follow-up of the six patients who responded, all the clinical and radiological findings regressed. CONCLUSION: In any patient with peripheral eosinophilia, abdominal pain and elevated liver enzymes, especially when CT reveals tubular and nodular hypodense lesions particularly in subcapsular area, F. hepatica infection should be considered. Either triclabendazole or bithionol can be used effectively for the treatment.     

Churg-Strauss syndrome presenting with acute myocarditis and cardiogenic shock

Churg-Strauss syndrome (CSS) is a multisystem disorder characterised by asthma, prominent peripheral blood eosinophilia, and vasculitis signs. We report the case of a 22 year-old man admitted to the intensive care unit for acute myocarditis complicated with cardiogenic shock. Eosinophilia, history of asthma, lung infiltrates, paranasal sinusitis, glomerulonephritis, and abdominal pain suggested the diagnosis of CSS. Cardiac MRI confirmed cardiac involvement with a diffuse subendocardial delayed enhancement of the left ventricular wall, and a left ventricular ejection fraction (LVEF) of 30%. Acute myocarditis was confirmed with myocardial biopsy. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, vasopressor inotropes, intra-aortic balloon pump and mechanical ventilation, and was discharged 21 days later. One year after diagnosis, the patient was asymptomatic. The eosinophilic cell count was normal. Follow-up MRI at one year showed LVEF of 40% with persistent delayed enhancement. Cardiac involvement by CSS requires immediate therapy with corticosteroids and cyclophosphamide, which may allow recovery of the cardiac function.     

Strongyloides stercoralis infection simulating polyarteritis nodosa

INTRODUCTION: Parasitic infection can present with many different clinical manifestations. EXEGESIS: A 77 year-old Russian woman, who's been living in France since 50 years was admitted for polyarthritis, myalgia, fever, abdominal pain, and eosinophilia simulating polyarterisis nodosa. Before admission, she was treated by steroids for polymyalgia rheumatica. The diagnosis of Strongyloides stercoralis was performed by parasitologic analysis of feces and colic biopsies. The outcome was favourable under treatment by ivermectine and steroid withdrawal. CONCLUSION: S. stercoralis can be associated with reactive arthritis. Case reports of S. stercoralis infection mimicking systemic vasculitis are exceptionnal.     

Hepatic vasculitis presenting with multiple sterile liver abscesses in a patient with systemic lupus erythematosus

Although biochemical evidence of liver disease is common in patients with systemic lupus erythematosus (SLE), clinical liver disease is uncommon. We report on a 26‐year‐old woman who presented with acute febrile illness, right upper abdominal pain and multiple hypo‐dense lesions in the liver in a computed tomographic study that mimicked multiple liver abscesses. Multiple necrotizing hepatic granulomas and old occlusive hepatic arteritis were observed in the surgical liver specimen. She was later found to have SLE. This patient represented a rare case of SLE that had hepatic vasculitis with hepatic infarction mimicking multiple liver abscesses as an initial manifestation.     

Eosinophilic cholecystitis along with pericarditis caused by Ascaris lumbricoides： A case report

Although the etiology of eosinophilic cholecystitis is still obscure, the postulated causes include allergies, parasites, hypereosinophilic syndrome, and eosinophilic gastroenteritis. It is sometimes accompanied by several complications, but a simultaneous onset with pericarditis is very rares. A 28-year-old woman complained of acute right hypocondrial pain and dyspnea associated with systemic eruption. Several imaging modalities revealed acute cholecystitis and pericarditis with massive pericardial effusion. A marked peripheral blood eosinophilia was observed, and the eruption was diagnosed as urticaria. Her serum had a high titer of antibody against Ascaris lumbricoides. Treatment with albendazole drastically improved all clinical manifestations along with normalization of the imaging features and eosinophilia. We report herein a rare case of simultaneous onset of acute cholecystitis and pericarditis associated with a marked eosinophilia caused by parasitic infection.     

Clinical features of clinically diagnosed eosinophilic liver abscesses

Purpose  

Eosinophilic liver abscesses (ELAs) are frequently encountered in the clinical field based on typical computed tomography (CT) findings and the presence of peripheral eosinophilia. In this study, the authors evaluated the clinical features and natural course of CT diagnosed ELAs.     

Biliary tract disease: a rare manifestation of eosinophilic gastroenteritis

Eosinophilic gastroenteritis (EGE) is a rare inflammatory disease characterized by diffuse or scattered eosinophilic infiltration of the digestive tract and usually by peripheral blood eosinophilia. The most common presenting symptoms of EGE are abdominal pain, vomiting and diarrhea, but clinical features depend on which layers or location of gastrointestinal tract are involved. Treatment with corticosteroids results in clinical and histological remission in most patients and surgery can be avoided if a correct diagnosis is made. Previous history of allergy is a key to diagnosing EGE, but peripheral eosinophilia may be absent in some patients under concomitant treatment with corticosteroids. Radiological and endoscopic findings are also nonspecific and diagnosis must always be histologically confirmed. The gastrointestinal involvement is patchy in distribution, so more than one panendoscopic examination is often necessary to establish the diagnosis, and surgical or CT-guided full-thickness biopsy is needed in patients with muscular or serosal involvement. It emphasises the importance of a high index of clinical suspicion, which mainly depends on knowledge of natural history of the disease. We report here a case of EGE associated with transmural eosinophilic cholecystocholangitis, in a patient who presented with dyspeptic symptoms and recurrent cholestasis responsive to corticoesteroids. To our knowledge, this patient represents the second case, in the English literature, in which corticoid-responsive cholangitis was associated to histologically proven eosinophilic cholecystitis and gastrointestinal involvement, suggesting that EGE must always be considered in the differential diagnosis of biliary tract disease in patients with eosinophilia and/or atopic diseases.     

Hypereosinophilic syndrome associated with antineutrophil cytoplasmic antibody]

A 68-year-old man was admitted to our hospital with complaints of fever, cough, and shortness of breath. He had several erythematous maculae on the trunk and experienced hypesthesia in his lower extremities. Laboratory data showed marked eosinophilia (20,235/mm3) and enhanced hepatobiliary enzymes. Chest X-ray films and computed tomographic scans revealed diffuse patchy infiltrative changes in the lungs. Histologic findings confirmed eosinophilic infiltration of the skin, liver, and lungs. A diagnosis of hypereosinophilic syndrome (HES) was made in accordance with clinical criteria proposed by Chusid et al. The patient was positive for antineutrophil cytoplasmic antibodies (a marker for vasculitis). This suggested a clinical picture resembling Churg-Strauss syndrome (CSS) despite the lack of bronchial asthma. The findings in this report could contribute to a better understanding of the diversity of HES cases, several of which are considered to represent a continuum of pathologies sharing an etiology similar to that of CSS.     

嗜酸细胞性胃肠炎的临床特征分析

背景:嗜酸细胞性胃肠炎(EG)的临床表现缺乏特异性,临床和病理医师对此尚未引起足够重视,诊断往往延迟。目的:探讨EG的临床特征。方法:回顾性分析2011年10月-2013年9月复旦大学附属中山医院确诊为EG患者的临床、实验室、内镜、影像学和治疗的资料。结果:10例EG患者的平均年龄41.9岁,4例有过敏史或哮喘史,起病至确诊时间平均25 d。临床均表现为腹痛,伴腹胀、腹泻或呕吐;8例血嗜酸性粒细胞增加,CT示7例胃和小肠壁广泛水肿,分层状肠壁增厚或腹腔积液。内镜检查发现6例胃窦、十二指肠或空肠黏膜充血糜烂。所有病例经胃肠黏膜活检和(或)腹水检查明确嗜酸性粒细胞浸润。7例患者予泼尼松治疗有效,1例停药后复发。结论:EG可能并非罕见,腹痛伴血嗜酸性粒细胞增多,或腹痛患者CT发现胃肠壁均匀水肿或分层状肠壁增厚,或伴腹水,需考虑EG,多部位包括十二指肠降部多点活检行病理学检查发现黏膜嗜酸性粒细胞浸润是确诊的主要手段。激素治疗可有效缓解症状和血嗜酸性粒细胞增多。     

Right hepatectomy for pyogenic liver abscesses with true multiloculation

We report the case of a 61-year-old woman with cryptogenic liver abscesses who had been profoundly ill with severe upper abdominal pain, impaired consciousness, prostration, continuous high fever secondary to sepsis, and thrombocytopenia (platelets, 1–5 × 10⁴/mm³) since admission. Ultrasonograms and computed tomograms revealed two separate multiloculated lesions in the right lobe of the liver, consistent with the liver abscesses. Immediately after diagnosis, percutaneous abscess drainage was performed under ultrasonographic guidance; however, only a small amount of pus was drained, prompting continuous irrigation of the abscess cavity. Four days later, transcatheter hepatic arterial infusion of antibiotics was attempted. However, the abscesses had enlarged and her general condition had worsened. On hospital day 8, she underwent right hepatectomy because the multiloculated lesions were refractory to drainage. The operation was successful in terms of hepatectomy, although she continued to suffer from sepsis, secondary right subphrenic abscess formation, and prolonged thrombocytopenia with associated coagulation disorders for two months. Examination of multiple cross sections of the resected specimen disclosed that the lesions consisted of aggregations of multiple small locules. There was no communication between the locules and there were true septations, rather than multiloculated lesions with pseudoseptations. The patient has been well for 2 years without recurrent abscess of the liver or any infectious disease.