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Background/aims: Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are the 3 main autoimmune liver diseases (AILDs). The epidemiology of AILD in Turkey is not known. To determine the scientific status, we performed a scientometric analysis of AILD-related original articles that originated from Turkey., Materials and Methods: We searched the Web of Science database, the Science Citation Index Expanded (SCI-E), and the Social Sciences Citation Index (SSCI) by using the keywords "autoimmune hepatitis," "primary biliary cholangitis/primary biliary cirrhosis," and "primary sclerosing cholangitis" in conjunction with "Turkey." A scientometric analysis was done on the search results., Results: We identified 117 AILD-related papers that were published in Turkey from 1997 to 2019. Among these, 70 case reports, letters, and reviews and 2 original articles that were not cited in SCI-E/SSCI were excluded. The remaining 45 original articles were further analyzed. These studies were related to AIH (n=22), PBC (n=7), PSC (n=9), PBC-AIH overlap (n=5), and others (n=2). Four of the publications originated in pediatric settings; 9 of 45 papers were published from 1997 to 2008 and 36 papers were published from 2009 to 2019. Most papers (75%) were reported from 5 centers; 9 papers (20%) were published in journals with an impact factor of 3 or higher., Conclusion: The overall number and quality of AILD-related papers in Turkey are unexpectedly low, although a number of papers have received considerable international recognition. More epidemiologic, prospective, and multicenter research projects are warranted to advance AILD knowledge and to produce high-quality research from Turkey.

Objective: Common Variable Immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency in adults. This study evaluates the clinical, immune, and immunohistological characteristics of hepatic involvement in CVID. Materials and Methods: Medical records of 72 CVID patients, including 7 with liver biopsies, were retrospectively reviewed. Immunohistochemical staining was performed to identify immune cell infiltration. Results: We enrolled 72 patients with a median age of 38 (22--77) years, and 47% (n = 34) were female. Elevated alkaline phosphatase (ALP) levels were found in 58.3% (n=42) of the patients. Hepatic involvement was more prevalent among females, and those with hepatic involvement displayed a higher incidence of autoimmune disease. Splenomegaly was identified in 85% of the patients with hepatic involvement. Notably, switched memory B cells (IgD- CD19+ CD27+) and the CD4+/CD8+ ratio were statistically lower in patients with hepatic involvement (p=0.031, p=0.047). Liver biopsies were performed on 7 patients, revealing minimal to severe chronic hepatitis. Immunohistologic analysis of these liver biopsies revealed varying degrees of positivity of CD8+, CD4+, and CD16+. However, staining for CD21, CD56, and CD138 yielded negative results. Conclusion: Our findings suggest that immune cells, particularly CD8+, CD4+, and CD16+ cells, may play a role in developing hepatic involvement. Understanding the underlying causes and mechanisms of hepatic involvement in CVID patients can lead to early diagnosis and targeted treatments, ultimately improving the prognosis. [ABSTRACT FROM AUTHOR]

Introduction: Rituximab, which is used in autoimmune rheumatic diseases (ARD), can cause both an increased risk of development of COVID‐19 disease and re‐infection due to its potent and long‐acting immunosuppression. So, we aimed to evaluate the frequency, risk factors and re‐infection rates of COVID‐19 in ARD patients receiving rituximab. Methods: A single‐center retrospective study was performed with patients receiving rituximab for ARD in 12 months before the onset of COVID‐19 in Turkey. The data regarding severe acute respiratory syndrome‐coronavirus 2 reverse transcription polymerized chain reaction (RT‐PCR) test, clinical, laboratory, and mortality data of all patients were collected from medical records. Logistic regression analysis was used for predictors of COVID‐19 disease. COVID‐19 re‐infection was defined as RT‐PCR positivity and recurrence of acute COVID‐19 symptoms after at least 1 negative RT‐PCR in patients with clinical improvement. Results: Ninety‐eight ARD patients with rituximab were evaluated and 23 (23%) of them had COVID‐19. The presence of hypogammaglobulinemia increased the risk of COVID‐19 disease 8‐fold. COVID‐19 pneumonia occurred in 13 (57%) and these patients' age was higher than those without pneumonia (59.6 ± 11.8 vs 44.9 ± 14.2 years, P = 0.013). Mortality due to COVID‐19 was 13% and COVID‐19 re‐infection was seen in 20% of survivors. Conclusion: Regardless of the underlying rheumatic disease and organ involvements, hypogammaglobulinemia in ARD could be a risk factor for COVID‐19 development, and advanced age could be for COVID‐19 severity. Moreover, COVID‐19 re‐infection rates are high. [ABSTRACT FROM AUTHOR]

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Introduction: Pemphigus refers to a group of rare autoimmune vesiculobullous diseases with high morbidity and mortality, mainly affecting the skin and the mucosae. Aim: To evaluate the incidence of pemphigus in the Eastern region of Turkey by analysing the clinical characteristics of the patients including the drugs used in the treatment and their side-effect profiles and dosing schedules, duration of treatment, length of hospital stay, and the characteristics of the lesions. Material and methods: The retrospective study included 130 pemphigus patients who were admitted to the inand out-patient dermatology clinics at the University Medical School between January 2005 and October 2017. Age, gender, length of hospital stay, localization of the lesions, initial and maintenance dosages, treatmentrelated complications, comorbidities, family history, and smoking status were recorded for each patient. Results: The 130 patients included 62 (47.69%) men and 68 (52.31%) women, of which 73 (56.15%) patients had a smoking history. The annual incidence rate of pemphigus was 1.036/100,000 population and pemphigus vulgaris (PV) was the most common clinical subtype of pemphigus in our patients (n = 122; 93.85%). The most common side effect of the treatment was oral candidiasis, followed by Cushingoid appearance. Conclusions: The results indicated that the incidence of pemphigus is remarkably high in the Eastern region of Turkey and the associated factors including lifestyle changes should be taken into mind when considering these patients. [ABSTRACT FROM AUTHOR]