Achilles tendonitis in a patient with Behçet's disease

A 56-year-old male patient had suffered from recurrent oral aphthae, acne-like rashes on the face, an erythema nodosum-like rash on the left lower leg, and severe heel pain on the left-hand side. Colonoscopy revealed six ulcerative lesions in the terminal ileum. Ultrasonography and magnetic resonance imaging showed an inflammatory lesion in the Achilles tendons, with greater inflammation on the left. Achilles tendonitis was considered to be a peripheral enthesopathy in this patient with Behçet's disease complicated by an ileal ulcer.     

Erosive arthritis in a patient with Behçet's disease

We report on a 35-year-old female patient suffering from Behçet's disease for 20 years who developed recurrent oral ulcerations during the onset of the disease. This was followed by recurrent genital ulcerations and joint symptoms involving knee, wrist and feet joints and bilateral heel pain. X-rays of the feet revealed erosive and destructive changes in the 1st metatarsophalangeal and 1st proximal interphalangeal joints of the right foot and enthesopathy on calcaneus. A literature review indicated that erosive arthritis is a rare event in Behçet's disease.     

Intestinal permeability and its association with the patient and disease characteristics in Crohn's disease

AIM:To assess the intestinal permeability (IP) in patients with Crohn's disease (CD) and study the association of IP with the patient and disease characteristics. METHODS: One hundred and twenty five consecutive patients of CD (Males: 66) were diagnosed on the basis of a combination of standard clinical, endoscopic, imaging and histological features. CD activity index (CDAI) was used to calculate the activity of the disease while the behavior of the disease was assessed by the modified Montreal classification. IP was measured by the ratio of the percentage excretion of ingested doses of lactulose and mannitol in urine (LMR). The upper limit of normality of LMR (0.037) was derived from 22 healthy controls. RESULTS: Thirty six percent of patients with CD had increased IP. There was no significant difference in mannitol excretion (patients vs controls = 12.5% vs 14.2%, P = 0.4652), but lactulose excretion was significantly higher in patients compared to healthy controls (patients vs controls = 0.326% vs 0.293%, P = 0.0391). The mean LMR was also significantly higher in the patients as compared to healthy controls [0.027 (0.0029-0.278) vs 0.0164 (0.0018-0.0548), P = 0.0044]. Male patients had a higher LMR compared to females [0.036 (95% CI 0.029, 0.046) vs 0.022 (95% CI 0.0178, 0.028) (P = 0.0024), though there was no difference in the number of patients with abnormal IP in boththe sexes. Patients with an ileo-colonic disease had a higher LMR than those with only colonic disease [0.045 (95% CI 0.033, 0.06) vs 0.021 (95% CI 0.017, 0.025) (P < 0.001)]. Of patients with ileo-colonic disease, 57.8% had an abnormal IP, compared to 26.7% with colonic and 15.6% with small intestinal disease. Patients with a stricturing disease had significantly higher LMR compared to non-fistulising non-stricturing disease [0.043 (95% CI 0.032, 0.058) vs 0.024 (95% CI 0.019, 0.029) (P = 0.0062)]. There was no correlation of IP with age, disease activity, duration of illness, D-xylose absorption, upper GI involvement, perianal disease, and extra- intestinal manifestations. On multiple regression analysis, male gender and ileo-colonic disease were independent factors associated with increased IP. Gender, location, behavior of the disease and upper GI involvement could explain up to 23% of variability in IP (R2 = 0.23). CONCLUSION: IP was increased in 36% of patients with CD. Male gender and an ileo-colonic disease were the independent factors associated with increased IP.     

Hemophagocytic syndrome in one patient with adult-onset Still’s disease

Macrophage activation syndrome (MAS) is an important complication seen in systemic for juvenile rheumatoid arthritis; until now, it has been reported in only a few cases of adult-onset Still’s disease (AOSD). Here, we shall present a 50-year-old female patient who was using steroids and antimalarial drugs for AOSD, and who developed MAS during follow-up. The patient presented with febrile neutropenia, and the neutropenic period lasted for 15 days. The examination of bone marrow aspiration smears demonstrated increased macrophages and findings of hemophagocytosis. Flow cytometric analysis of peripheral blood showed decreased natural killer cells. The patient developed neurologic findings during this period, and during the recovery of neutropenia, she had icterus and liver function test abnormalities. The patient was given granulocyte colony-stimulating factor during neutropenic period, and her neutropenia improved after the administration of high-dose steroids. Our patient was the first AOSD patient who presented with febrile neutropenia during the course of her disease and who was diagnosed to have MAS.     

Catastrophic antiphospholipid syndrome in a patient with Behçet's disease

We report on a patient who had a life-threatening relapse of Beh?et's disease associated with a catastrophic antiphospholipid syndrome. The patient experienced over a short time a recurrent acute myocardial infarction, multiple venous thromboses, uveitis, and erythema nodosum. Search for thrombophilic factors was positive only for lupus anticoagulant (LAC) and criteria for the diagnosis of the antiphospholipid antibody syndrome were fulfilled. LAC was not found three months after the discharge. At that time the patient had no evidence of clinically active disease or thrombosis. We suggest that LAC was the main triggering factor for the repeated thromboses in this patient.     

Cutaneous polyarteritis nodosa in a patient with Crohn’s disease

A 19-year-old Japanese woman with a 4-year history of Crohn’s disease (CD) developed high fever, polyarthralgia, and painful subcutaneous nodules of the legs. A skin biopsy showed panarteritis with fibrinoid necrosis in the deep dermis. Endoscopic examination showed aphthous lesions in the entire colon. She was diagnosed with cutaneous polyarteritis nodosa (PAN) associated with CD. Steroid therapy improved her symptoms. To our knowledge, this is the first Japanese case of cutaneous PAN associated with CD.     

Virtual colonoscopy-induced perforation in a patient with Crohn＇s disease

We report a case of sigmoid colon perforation in a patient with Crohn's disease undergoing computed-tomographic (CT) colonography. A 70-year-old patient with Crohn's disease with terminal ileitis and sigmoid stricture underwent CT colonography after incomplete conventional colonoscopy. During the procedure, the colon was inflated by air insufflation and the patient developed abdominal pain with radiological evidence of retroperitoneal and intraperitoneal free gas. Hartmann's operation was performed. This case highlights that CT colonography is not risk-free. The risk of perforation may be higher in patients with inflammatory bowel disease.     

EBV-associated haemophagocytic syndrome in a patient with Behçet's disease

We present a case of Epstein-Barr virus (EBV)-associated haemophagocytic syndrome in a patient with Beh?et's disease. A 43-year-old man, who had been receiving treatment under the diagnosis of Beh?et's disease for recurrent oral ulcers, genital ulcer, ileal ulcer, and arthritis, had been admitted for fever, headache, and nausea developed 3 days ago. Laboratory data showed pancytopaenia, an increase in liver enzymes, lactate dehydrogenase (LDH) and ferritin. Haemophagocytic syndrome was diagnosed from histiocytosis and haemophagocytosis by macrophages, shown in the bone marrow aspiration and biopsy, and in situ hybridization for EBV showed a positive finding. The patient recovered rapidly after steroid therapy. This is the first report of EBV-associated haemophagocytic syndrome developed in a patient with Beh?et's disease.     

Intestinal permeability and its association with the patient and disease characteristics in Crohn＇s disease

AIM：To assess the intestinal permeability （IP） in patients with Crohn＇s disease （CD） and study the association of IP with the patient and disease characteristics.
METHODS： One hundred and twenty five consecutive patients of CD （Males： 66） were diagnosed on the basis of a combination of standard clinical, endoscopic, imaging and histological features. CD activity index （CDAI） was used to calculate the activity of the disease while the behavior of the disease was assessed by the modified Montreal classification. IP was measured by the ratio of the percentage excretion of ingested doses of lactulose and mannitol in urine （LMR）. The upper limit of normality of LMR （0.037） was derived from 22 healthy controls.
RESULTS： Thirty six percent of patients with CD had increased IP. There was no significant difference in mannitol excretion （patients vs controls = 12.5% vs 14.2%, P = 0.4652）, but lactulose excretion was significantly higher in patients compared to healthy controls （patients vs controls = 0.326% vs 0.293%, P = 0.0391）. The mean LMR was also significantly higher in the patients as compared to healthy controls [0.027 （0.0029-0.278） vs 0.0164 （0.0018-0.0548）, P = 0.0044]. Male patients had a higher LMR compared to females [0.036 （95% CI 0.029, 0.046） vs 0.022 （95% CI 0.0178, 0.028） （P = 0.0024）, though there was no difference in the number of patients with abnormal IP in boththe sexes. Patients with an ileo-colonic disease had a higher LMR than those with only colonic disease [0.045 （95% CI 0.033, 0.06） vs 0.021 （95% CI 0.017, 0.025） （P 〈 0.001）]. Of patients with ileo-colonic disease, 57.8% had an abnormal IP, compared to 26.7% with colonic and 15.6% with small intestinal disease. Patients with a stricturing disease had significantly higher LMR compared to non-fistulising non-stricturing disease [0.043 （95% CI 0.032, 0.058） vs 0.024 （95% CI 0.019, 0.029） （P = 0.0062）]. There was no correlation of IP with age, disease activ     

Ustekinumab in a patient with Crohn's disease and anti-TNF-α-induced psoriasis

Treatment with anti-tumor necrosis factor (TNF)-α for Crohn's disease is relatively safe, although various cutaneous adverse effects have been reported such as the development or exacerbation of anti-TNF- α-induced psoriasis, which can sometimes lead to treatment withdrawal. Therefore, new alternative treatments with new mechanisms of action are required. Ustekinumab, a monoclonal antibody against the p40 subunit of interleukin 12/23, could induce response in patients with Crohn's disease and has demonstrated efficacy in patients with psoriasis. We present the case of a woman with Crohn's disease who developed psoriasis after treatment with two anti-TNF- α drugs (infliximab and adalimumab). The patient was subsequently treated with ustekinumab with resolution or psoriasis lesions and maintenance of remission of Crohn's disease.     

Bronchiolitis obliterans organizing pneumonia in a patient with Behçet's disease

A 55-year-old male presented a productive cough, haemoptysis, and intermittent fever for 2 weeks. He was diagnosed as having Behcet's disease (BD) with manifestations of recurrent oral ulcers, genital ulcers, erythema nodosum, a gastro/duodenal ulcer, colon ulcers, and folliculitis. After admission, a chest X-ray showed bilateral ill-defined patches. Subsequent chest computerized tomography revealed multiple patchy consolidations over the left and right lower lobes. Because of poor response to oxacillin antibiotic treatment, an open lung biopsy was arranged. Bronchiolitis obliterans organizing pneumonia (BOOP) was diagnosed. Methylprednisolone pulse therapy, followed by prednisolone and cyclophosphamide maintenance, was prescribed. The patient responded well, with clinical and radiological resolution. Early diagnosis with proper treatment is crucial in the management of this lethal pulmonary disease.     

Severe cholestasis due to adalimumab in a Crohn’s disease patient

Elevation of liver biochemistry has been reported with anti-tumor necrosis factor agents, but overt liver failure rarely reported. Autoimmune hepatitis has been more commonly reported with infliximab than adalimumab(ADA). Our case, however, describes the first reported case of ADA-associated severe cholestatic injury. A 39-year-old female with Crohn’s disease developed severe jaundice after initiation of ADA. All serologic tests and imaging studies were normal. Liver biopsy showed prominent pericentral canalicular cholestasis,without features of steatosis or sclerosing cholangitis,consistent with drug-induced cholestasis. The serum total bilirubin peaked at 280 μmol/L, and improvement was seen after 5 wk with eventual normalization of liver enzymes at 10 wk. Our case describes the first reported case of ADA-associated severe cholestatic liver disease and the first histopathologic examination of this adverse drug effect. Clinicians need to be aware of this potential drug-induced liver injury when prescribing this commonly used biologic medication.     

Leprosy mimics adult onset Still’s disease in a Chinese patient

Background

Leprosy is an infectious disease that mainly involves the skin and peripheral nerves. It is caused by acid-fast, rod-shaped bacillus Mycobacterium leprae. In recent time, it has become a major global health concern which requires an early diagnosis and proper treatment in order to avoid lifelong neuropathy and disability. It is considered a ‘great mimicker’ as it exhibits a similar clinical features that resembles many connective tissue disease.

Cyclosporine A-induced neck fibrosis in a patient with adult-onset Still’s disease

Cyclosporine A (CsA) is an immunosuppressive agent used for the prevention of graft rejection during organ and bone marrow transplantation. CsA is also used for the treatment of various inflammatory rheumatic diseases. Although different side effect profiles have been reported, nephrotoxicity, renal vascular damage, hypertension, and gingival hypertrophy are among the most commonly encountered side effects. The development of massive fibrosis in the neck associated with CsA in a 30-year-old male patient with Still’s disease is presented herein. Significant regression was observed after the discontinuation of CsA.