Classifying Lupus Nephritis: An Ongoing Story

The role of the renal biopsy in lupus nephritis is to provide the diagnosis and to define the parameters of prognostic and therapeutic significance for an effective clinicopathological correlation. Various classification schemas initiated by World Health Organization in 1974 have been proposed until the most recent update by International Society of Nephrology/Renal Pathology Society in 2004. In this paper, we reviewed the new classification system with the associated literature to highlight the benefits and the weak points that emerged so far. The great advantage of the classification emerged to provide a uniform reporting for lupus nephritis all over the world. It has provided more reproducible results from different centers. However, the studies indicated that the presence of glomerular necrotizing lesion was no longer significant to determine the classes of lupus nephritis leading to loss of pathogenetic diversity of the classes. Another weakness of the classification that also emerged in time was the lack of discussions related to the prognostic significance of tubulointerstitial involvement which was not included in the classification. Therefore, the pathogenetic diversity of the classification still needs to be clarified by additional studies, and it needs to be improved by the inclusion of the tubulointerstitial lesions related to prognosis

MEFV gene mutations in Henoch- Schonlein purpura

ensari, arzu/0000-0001-7036-4457WOS: 000334521000017PubMed: 23981758AimCoexistence of familial Mediterranean fever (FMF) with various systemic vasculitides, including Henoch-Schonlein purpura (HSP) and other inflammatory disorders has been reported and the MEFV gene has been suggested to play an important role in the pathogenesis of this association. In the present study, the mutation rate of the MEFV gene in HSP and its association with the clinical course of the disease were evaluated. MethodThe study group comprised 68 children (36 boys and 32 girls) diagnosed as having HSP. The spectrum and degree of organ involvement and the levels of serum C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were documented for each patient. Allele-specific PCR using oligonucleotide probes which include 12 MEFV mutations (E148Q, P369S, F479L, M680I [G/C], M680I [G/A], I692del, M694V, M694I, K695R, V726A, A744S, R761H) were used for mutation analysis. ResultsOf the 68 patients studied, 50 (74%) showed no mutation, while 18 (26%) had MEFV mutation. Mutation analysis of the whole group revealed that 15 (22%) patients were heterozygous for one of the screened MEFV mutations, while three (4.5%) patients were compound heterozygous for two of the studied mutations, and one (1.5%) patient was homozygous for E148Q/E148Q mutations. Gastrointestinal and joint involvement, and edema were more frequently observed in patients with MEFV mutations, while ESR and CRP levels were significantly higher (P<0.05) in patients with MEFV mutations. ConclusionMEFV mutations, especially, E148Q and M694V, mutations might be associated with HSP and may affect clinical presentation and laboratory findings in HSP patients

Collagenous colitis in a patient with systemic sclerosis: a rare entity.

Collagenous colitis has been associated with autoimmune diseases. Co-occurence of systemic sclerosis and collagenous colitis is particularly rare. Herein, we described a 65-year-old woman with systemic sclerosis whose diarrhea and abdominal cramping were due to collagenous colitis. We have reviewed the clinical and histopathological features of collagenous colitis with regard to its concomitance with systemic sclerosis

The coexistence of focal segmental glomerulosclerosis with carcinoid syndrome: A case report

Carcinoid tumor is originated from neuroendocrine tumorfamily and a rarely presents in humans. Membranous andmembrano-proliferative glomerulonephritis were rarelyreported with presence of carcinoid tumors. However,the coexistence of focal segmental glomerulosclerosis(FSGS) with carcinoid syndrome have not been definedpreviously. We herein present a firs case report of FSGSduring follow up of carcinoid syndrome.Key words: Carcinoid sydrome, focal segmental glomerulosclerosis,glomerulonephriti

Sarcoidosis, Celiac Disease and Deep Venous Thrombosis: a Rare Association

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology and it may rarely be associated with a second disorder. Celiac disease is an immune-mediated enteropathy characterized with malabsorption caused by gluten intolerance, and several reports indicate an association between celiac disease and sarcoidosis. In addition, although celiac disease is associated with several extraintestinal pathologies, venous thrombosis has been rarely reported. Herein we present a rare case report of a patient with a diagnosis of sarcoidosis, celiac disease and deep venous thrombosis because of the rare association of these disorders. The patient was admitted with abdominal pain, weight loss, chronic diarrhea and a 5-day history of swelling in her right leg. A diagnosis of deep venous thrombosis was achieved by doppler ultrasonographic examination. The diagnosis of celiac disease was made by biopsy of duodenal mucosa and supported with elevated serum level of anti-gliadin IgA and IgG, and a diagnosis of sarcoidosis was achieved by transbronchial needle aspiration from the subcarinal lymph node during flexible bronchoscopy

Türk çocuklarında çölyak hastalığının erken tanısı için immünokromatografik çizgi yöntemiyle insan doku transglutaminazına karşı antikor taraması

Amaç: Çölyak hastalığı tüm dünyada yaygın sıklıkta görülmektedir. Türkiye?de dünya ile karşılaştırılabilecek epidemiyolojik veriler sınırlı sayıdadır. Bu ön hazırlayıcı çalışmanın amacı, Türk çocuklarında çölyak hastalığı sıklığını 1000 olguda yeni, kolay ve görsel tek adımda yapılabilen bir immünolojik tarama yöntemiyle belirlemektir. Yöntem: Bu prospektif çalı şma, Ankara Üniversitesi Tıp Fakültesi Çocuk Polikliniğine başvuran, sağlıklı görünen veya çölyak hastalığı dışında bir rahatsı zlığı olan, 2-18 yaş arası 1000 çocuk ve ergenden bilgilendirilmiş onam formu ile gönüllü olarak alınan serum örneklerinde yapıldı. Serumlar insan doku transglutaminazına ve gliadine karşı oluşmuş IgA sınıfı antikorlar için hızlı, görsel immünokromatografik çölyak hastalığı çizgisel immun yöntemiyle çalışıldı. Tarama testi sonuçları pozitif olan serumlar ikinci basamak doğrulama aşaması için ELISA yöntemi ile insan doku transglutaminazına karşı oluşmuş endomisyal antikor IgA ve antigliadin antikorlar IgA/IgG için ayrıca test edildiler. Anti doku transglutaminaz ve/veya endomisyal antikor pozitif olan bütün çocuklara ince barsak biyopsisi önerildi. Bulgular: 1000 olgunun 10?unda (%1) insan doku transglutaminazına karşı pozitif antikor tarama sonucu saptandı. Bütün doku transglutaminaz antikoru pozitif örnekler ELISA yöntemiyle endomisyal antikor çalışıldığında uyumlu görüldüler. Takiben yapılan ince barsak biyopsisinde de biri hariç dokuz olguda çölyak hastalığı tanısı doğrulandı. Biyopsi ile kanıtlanmış çölyak hastalığı prevalansı 1/111 (%0,9) olarak saptandı. Sonuçlar: Çölyak hastalığı için anti-doku transglutaminaz antikorlarının kolay görsel bir yöntemle belirlenmesi ELISA yöntemi kadar güvenilirdir. Yapması ve yorumlaması kolay bu testin, ucuz ve hızlı bir tarama yöntemi olarak geniş toplum tabanlı çalışmalarda uygulanabileceği düşünüldü. Bu ön çalışmada Türk çocukları örnek grubunda saptanan çölyak hastalığı sıklığı (1:111 veya %0.9) Avrupa, Orta Doğu ülkeleri ve ABD verileri ile benzer bulundu.Background/aims: Celiac disease has a large prevalence worldwide. There are a limited number of comparable epidemiological data for celiac disease in Turkey. The aim of this preliminary study was to determine the prevalence of celiac disease in a sample of 1000 Turkish children by a novel, simple, and visual one-step immunoassay screening test. Methods: This prospective study consisted of 1000 serum samples from apparently healthy children and children with disorders other than celiac disease aged between 2-18 years who presented as outpatients at Ankara University, Faculty of Medicine, Department of Pediatrics. Sera were tested for IgA-class antibodies against human tissue transglutaminase and gliadin by rapid immunochromatographic line immunoassay. Endomysial antibody IgA against human tissue transglutaminase and AGA IgA/IgG were also tested by ELISA as a second step when the result of the screening test was positive. Small bowel biopsy was recommended to all the children with positive anti-tissue transglutaminase and/or endomysial antibody results. Results: Ten of the 1000 individuals (1%) had positive antibody screening test to human tissue transglutaminase. All tissue transglutaminase-positive samples revealed good correlation with endomysial antibody by ELISA method. Subsequently small bowel biopsy was performed in all serology-positive cases. Biopsy results confirmed a diagnosis of celiac disease in nine cases. The prevalence of biopsy- proven celiac disease was 1:111 (0.9%). Conclusions: Determination of anti-tissue transglutaminase antibodies by simple visual system for celiac disease appeared to be as reliable as the ELISA system. It is easy to perform and interpret, costeffective, and rapid, as pointed out in other previous studies, as a screening test in large population-based studies. The prevalence of celiac disease in the overall sample of Turkish children (1:111 or 0.9%) in this preliminary study is similar to that reported in European and Middle Eastern countries and the United States

Cytomegalovirus Infection in a Patient with Crohn’s Ileocolitis

Cytomegaloviral enterocolitis is an uncommon infection that can complicate inflammatory bowel disease. A case of a patient with a three-year history of Crohn's disease is reported. He had been in a stable condition on mesalamine 4 g/day and methylprednisolone 10 mg/day for three years until four weeks before admission. The patient was admitted with complaints of fever, abdominal pain and watery diarrhea. A diagnosis of an exacerbation of Crohn's disease was established. The radiological examination revealed narrowing of the terminal ileum. Multiple fistulas and abscess-like images were observed. The patient then underwent ileocolic resection and ileostomy. The histopathological examination revealed Crohn's ileocolitis with superimposed cytomegalovirus infection. In patients with rapidly deteriorating inflammatory bowel disease, cytomegalovirus infection should be kept in mind as one of the differential diagnoses

Human tissue transglutaminase antibody screening by immunochromatographic line immunoassay for early diagnosis of celiac disease in Turkish children

ensari, arzu/0000-0001-7036-4457; Kuloglu, Zarife/0000-0001-9442-7790WOS: 000254611300004PubMed: 18386235Background/aims: Celiac disease has a large prevalence worldwide. There are a limited number of comparable epidemiological data for celiac disease in Turkey. The aim of this preliminary study was to determine the prevalence of celiac disease in a sample of 1000 Turkish children by a novel, simple, and visual one-step immunoassay screening test. Methods: This prospective study consisted of 1000 serum samples from apparently healthy children and children with disorders other than celiac disease aged between 2-18 years who presented as outpatients at Ankara University, Faculty of Medicine, Department of Pediatrics. Sera were tested for IgA-class antibodies against human tissue transglutaminase and gliadin by rapid immunochromatographic line immunoassay. Endomysial antibody IgA against human tissue transglutaminase and AGA IgA/IgG were also tested by ELISA as a second step when the result of the screening test was positive. Small bowel biopsy was recommended to all the children with positive anti-tissue transglutaminase and/or endomysial antibody results. Results: Ten of the 1000 individuals (1%) had positive antibody screening test to human tissue transglutaminase. All tissue transglutaminase-positive samples revealed good correlation with endomysial antibody by ELISA method. Subsequently small bowel biopsy was performed in all serology-positive cases. Biopsy results confirmed a diagnosis of celiac disease in nine cases. The prevalence of biopsy-proven celiac disease was 1:111 (0.9%). Conclusions: Determination of anti-tissue transglutaminase antibodies by simple visual system for celiac disease appeared to be as reliable as the ELISA system. It is easy to perform and interpret, cost-effective, and rapid, as pointed out in other previous studies, as a screening test in large population-based studies. The prevalence of celiac disease in the overall sample of Turkish children (1:111 or 0.9%) in this preliminary study is similar to that reported in European and Middle Eastern countries and the United States