A brief communication of patients with homozygous C282Y mutation-related hereditary hemochromatosis.

Hereditary hemochromatosis (HH) is an autosomal recessive inherited iron-loading disorder and is characterized by chronic hepatitis, cirrhosis, diabetes, and bronze skin. The hemochromatosis gene (C282Y homozygosity)-related hemochromatosis is the most common form of HH. The prevalence of HH is varied.

Clinical and serological characteristics of anti-Ro/SS-A and anti-La/SS-B negative primary Sjögren's syndrome: a comparative study.

Objective: This study aimed to describe the clinical spectrum of primary Sjögren's syndrome (pSS) patients with anti-Ro/SS-A and anti-La/SS-B negativity.

Patients And Methods: From a single-center study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, those with triple seronegativity anti-Ro/SS-A (anti-Sjögren's-syndrome-related antigen A autoantibody), anti-La/SS-B (anti-Sjögren's-syndrome-related antigen B autoantibody), rheumatoid factor (RF) (-) and antinuclear antibody (ANA)(+)] or [anti-Ro/SS-A(-), anti-La/ SS-B(-), RF(+) and ANA(-)] and quad¬ruple seronegativity [anti-Ro/SS-A(-), anti-La/SS-B(-), RF(-) and ANA(-)] were identified retrospectively. Clinical, serological, and laboratory features were compared.

Poor obstetric outcomes in women with takayasu arteritis: a retrospective cohort study.

The objective of this study was to assess the pregnancy outcomes in a cohort of patients who experienced pregnancies before and/or after being diagnosed with Takayasu's arteritis (TA). The present investigation encompassed a total of 88 pregnancies seen in a cohort of 35 patients who met the criteria outlined by the American College of Rheumatology in 1990 for the classification of Takayasu arteritis (TA). Pregnancies were classified into two categories.

Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study.

Objective: Single-organ cutaneous small-vessel vasculitis (SoCSVV) is an inflammatory skin-limited vascular disease affecting the dermal and/or hypodermal vessel wall. Pathogenetically, idiopathic forms are described, as well as the induction from different triggers, such as infections, drugs, and vaccines. Following the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic outbreak, cases of cutaneous vasculitis induced by both COVID-19 and COVID-19 vaccinations have been reported in literature.

A combination of non-invasive tests for the detection of significant fibrosis in patients with metabolic dysfunction-associated steatotic liver disease is not superior to magnetic resonance elastography alone.

Objectives: The aims of the present study were to investigate a combination of magnetic resonance elastography (MRE) and vibration-controlled transient elastography (VCTE) or MRE and fibrosis score 4 (FIB-4) in the detection of significant fibrosis in patients with metabolic dysfunction-associated steatotic liver disease (MASLD).

Methods: Between November 5, 2021, and March 4, 2022, a total of 119 consecutive patients with MASLD were included. Liver stiffness was measured using liver biopsy, MRE, VCTE, and FIB-4.

Management of Biliary Complications in Liver Transplant Recipients with Duct-To-Duct Anastomosis: A Single-Center Experience.

Background: The aims of this study were to investigate biliary complications in liver transplant recipients with choledochocholedocho stomy anastomosis, to identify the risk factors for the development of such complications, and to evaluate the success of endoscopic approaches in liver transplant recipients.

Methods: Between January 2013 and May 2021, a total of 238 patients with liver diseases underwent liver transplantation: 174 recipients undergoing choledochocholedochostomy anastomosis were included in the analysis.

Results: Their median age was 54.

The Impact of Inflammatory Bowel Diseases and Related Medications on COVID-19 Severity and Outcome: A Tertiary Referral Center Experience from Turkey.

Background: Coronavirus disease-2019 has become a serious pandemic, and still remains a risk despite vaccines that have been devel- oped. Among inflammatory bowel disease patients old age, inflammatory bowel disease activation, the existence of the comorbid dis- ease, and using steroids are known risk factors for severe coronavirus disease-2019. But there are different data for drugs other than corticosteroids used.

COVID-19 Among Patients With Inflammatory Rheumatic Diseases.

Background: The course of novel coronavirus disease 2019 (COVID-19) has been of special concern in patients with inflammatory rheumatic diseases (IRDs) due to the immune dysregulation that may be associated with these diseases and the medications used for IRDs, that may affect innate immune responses.

Objective: In this cohort study, we aimed to report the disease characteristics and variables associated with COVID-19 outcome among Turkish patients with IRDs.

Methods: Between April and June, 2020, 167 adult IRD patients with COVID-19 were registered from 31 centers in 14 cities in Turkey.

Phenotypic characterization of Familial Mediterranean Fever patients harboring variants of uncertain significance.

Background/aim: Familial Mediterranean Fever (FMF) is the prototype of hereditary autoinflammatory disorders and caused by mutations on the MEFV gene located on the short arm of chromosome 16. Although some MEFV variants are clearly associated with disease phenotype, there are numerous variants with unknown clinical association which are termed as variants of uncertain significance (VUS). Here, we present clinical correlations of VUS in a large cohort of adult FMF patients from three tertiary centers located in Central Anatolia.

Appendectomy history is associated with severe disease and colchicine resistance in adult familial Mediterranean fever patients.

Background/aim: Peritonitis attacks of Familial Mediterranean Fever (FMF) usually requires emergency medical admissions and it’s hard to distinguish a typical abdominal attack from surgical causes of acute abdomen. Therefore, history of abdominal surgery, particularly appendectomy, is very common in patients with FMF. However, history of appendectomy might also give some clues about the course of FMF in the adulthood.

Predictors of persistent inflammation in familial Mediterranean fever and association with damage.

Objective: Persistent inflammation is an insidious and less studied feature of FMF. We investigated clinical determinants of persistent inflammation and its associations with individual damage items.

Methods: This is a cross-sectional analysis of 917 FMF patients, who fulfilled the Tel Hashomer criteria and had at least 6 months' follow-up.

Comparison of clinical features in FMF patients according to severity scores: An analysis with the ISSF scoring system.

Objective: Familial Mediterranean fever (FMF), is an auto-inflammatory disease characterized by attacks of fever and serositis. Some scoring systems have been developed to evaluate the severity of the disease, however, predicting the severity of FMF is not possible with current knowledge. Our aim in this study was to evaluate the factors affecting disease severity in FMF.

Factors associated with damage in patients with familial Mediterranean fever.

Objectives: Defining predictors of damage would improve patient care. We applied damage indexes to patients with familial Mediterranean fever (FMF) and identified the predictors of damage.

Methods: This is a cross-sectional analysis of 926 FMF patients, who fulfilled the Tel-Hashomer criteria and had at least six months of follow-up.

Colchicine intolerance in FMF patients and primary obstacles for optimal dosing.

Background/aim: Colchicine is the mainstay of treatment in FMF. However, in daily practice it is not easy to maintain effective colchicine doses in a substantial number of patients due to its side effects. In this study, we aimed to investigate prevalence and risk factors for colchicine side effects that limit optimal drug dosing and cause permanent discontinuation.

Familial Mediterranean fever-associated infertility and underlying factors.

Introduction/objectives: Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever, serositis, and arthritis, but some patients may experience long-term complications of disease such as infertility/subfertility. The published data about FMF-associated infertility is still limited. The aim of this study is to investigate the frequency and to determine potential factors for FMF-associated infertility/subfertility.

Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study.

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort.

Dose-dependent bradycardia as a rare side effect of corticosteroids: a case report and review of the literature.

Corticosteroids rank at the first place among the most commonly used immunosuppressive agents in the rheumatology practice. Although their conventional adverse effects including hyperglycemia, hypertension, hyperlipidemia, and osteoporosis are well-recognized and managed, steroid-induced cardiac arrhythmias are known to a lesser extent. In this regard, steroid-associated bradycardia is rarely expected and not very well known.

How should we manage low-dose methotrexate-induced pancytopenia in patients with rheumatoid arthritis?

Low-dose methotrexate (ld-MTX) that is administered during rheumatoid arthritis (RA) treatment has hematological adverse effects such as pancytopenia, although rare. Although well-established and widely used for hematological adverse effects caused by high-dose MTX, leucovorin (folinic acid) treatment does not have an agreed-upon administration for ld-MTX-induced pancytopenia. Here, we aimed to figure out whether there was any difference in response time between the regimens with and without folinic acid prescribed to our patients who developed pancytopenia while on MTX therapy, and to identify risk factors for its development.

Can neck pain be an initial symptom of rheumatoid arthritis? A case report and literature review.

Although rheumatoid arthritis (RA) is most commonly associated with peripheral joints, cervical spine involvement can be seen in almost 80% of patients in the presence of long-term disease, joint erosion, and risk factors such as male sex and rheumatoid factor positivity. It is very rare to have cervical involvement in the initial period of RA. If a patient has isolated cervical spine involvement without peripheral arthritis, it is highly likely that inappropriate investigations and delayed treatment may occur.

An incidental case of biliary fascioliasis mimicking cholangiocellular carcinoma.

Fascioliasis is a zoonotic infestation caused by Fasciola hepatica that usually attacks mammals, such as goats, sheep and cattle. The parasites can infect humans via freshwater plants contaminated with encysted metacercariae. In the acute phase, which involves hepatic invasion, the disease may present with abdominal pain, mild fever and hepatomegaly.

Idiopathic thrombocytopenic purpura associated with splenic tuberculosis: case report.

Tuberculosis is still one of the most prevalent and fatal infectious diseases in spite of considerable improvements in medical science. Splenic tuberculosis is a rare form of extrapulmonary tuberculosis. There are limited numbers of cases in which immune thrombocytopenia is associated with splenic tuberculosis.