A case of primary Sjögren’s syndrome with pulmonary-limited Wegener’s granulomatosis

A 60-year-old woman had a history of dyspnea for 5–6 weeks. The chest radiograph and computed tomography scans revealed bilateral patchy reticulonodular pattern. The patient had positive test results for antineutrophil cytoplasmic antibody against proteinase-3 (c-ANCA), antinuclear antibody and anti-Ro antibody. According to European Study Group on Classification Criteria for Sjögren’s Syndrome, the patient was diagnosed as primary Sjögren’s syndrome based on the presence of clinical features, positive findings on Schirmer’s test and parotis scintigraphy. Lung biopsy obtained by wedge resection showed granulomatous inflammation with extensive multinuclear giant cells involving the lung parenchyma and vascular structures. There was neither upper airway nor renal involvement. Thus, the patient was simultaneously diagnosed as pulmonary-limited Wegener’s granulomatosis. With this unique case, we would like to emphasize that the awareness of ANCA-associated vasculitis as a diagnostic possibility in primary Sjögren’s syndrome is important during the work-up of lung lesions.     

Cutaneous alternariosis in a patient with systemic lupus erythematosus

Alternaria species are common saprophytic fungi that naturally subsist on decaying plant materials, and occasionally may cause diseases in human beings and domestic animals. They can be a potential opportunistic pathogen in immunocompromized hosts or those with significant underlying disease. However, rarely they are also pathogen in otherwise healthy hosts. We report here the first case of cutaneous alternariosis in a 30-year-old woman who was on systemic steroid therapy for active systemic lupus erythematosus. The patient was referred to our department with purple papules and ulcerated nodules on the dorsum of the hands, wrists and ankles. Skin biopsy showed granulomatous reaction with fungal elements that were subsequently identified as Alternaria species. Individual lesions were successfully treated with oral itracanozole 200mg daily for six weeks. Besides the patient's own disease, the use of systemic steroid use might be a possible predisposing factor for the development of cutaneous alternariosis.     

The factors considered as trigger for the attacks in patients with familial Mediterranean fever

Although the inflammatory cascade of familial Mediterranean fever (FMF) is partially understood, triggering factors of those attacks has not been studied well. It is supposed that physical stresses such as cold exposure, tiredness and emotional stresses could provoke attacks. This study is aimed to survey the factors regarded as triggering the attacks in patients with FMF and their relationship with MEFV gene mutations. Clinical findings and genetic mutations (consist of M694V, M694I, M680I, V726A, E148Q) of patients were recorded. Patients were questioned about cold exposure, emotional stress, tiredness, long-lasting standing, long-duration travel, starvation, high intake of food, trauma, and infection as triggering factors for the attacks with both serositis and musculoskeletal pain. The study is comprised of 275 FMF patients (male/female: 177/98). The most common triggering factors for the attacks with serositis were cold exposure (59.3 %), emotional stress (49.8 %), tiredness (40.0 %) and menstruation (33.7 % in females). Long-lasting standing (78.8 %), long-duration travel (64.1 %) and tiredness (47.8 %) were the triggering factors for the attacks with musculoskeletal symptoms. The relationships between MEFV mutations and triggering factors were found as M694V allele with starvation, E148Q allele with high intake of food and V726A allele with long-duration travel. The attacks with serositis seem to be triggered by those factors to which whole body exposed, whereas the attacks with musculoskeletal complaints seem to be triggered by those factors to which regional or local part of body exposed. Since the number of alleles was small, a clear conclusion for a relationship between a particular gene variant and a specific trigger was not made.     

Clinical importance of antineutrophil cytoplasmic antibody positivity during propylthiouracil treatment

Background: Propylthiouracil (PTU) is the mainstay of antithyroid drug therapy. Previous studies reported antineutrophil cytoplasmic antibody (ANCA)‐positive vasculitis in patients treated for Graves’ disease. ANCA has been associated with either PTU or to the disease itself. However, this issue has not been investigated in toxic multinodular goitre (TMNG). The aim of this study was to evaluate the frequency of ANCA positivity in both TMNG and Graves’ disease patients treated with PTU, and to investigate the clinical importance of this issue. Patients and methods: We studied the presence of ANCA in 46 patients treated with PTU (30 Graves’ disease, 16 TMNG). Two years after the discontinuation of PTU, ANCA was re‐evaluated in 29 patients (18 Graves’ disease, 11 TMNG). Results: By indirect immunofluorescence, 19 of the 46 patients (41.3%) on PTU treatment were ANCA positive [13 of the 30 patients in Graves disease (43.3%), six of the 16 patients in TMNG (37.5%)]. There was no statistically significant difference between Graves’ disease and TMNG patients for ANCA positivity (p = 0.362). ANCA positivity was not related to gender, thyroid autoantibodies, alanine aminotransferase, aspartate aminotransferase, neutrophil count and PTU dose. Two years after withdrawal of PTU treatment, 10.3% of patients continued to have positive ANCA (p < 0.0001). Signs and symptoms of vasculitis could not be detected in any of the ANCA‐positive patients. Conclusion: Our study suggests that PTU but not Graves’ disease itself is the most important factor for ANCA development. The frequency of ANCA positivity is 41.3% in our country which was not different in Graves’ disease and TMNG patients. The dose of PTU and ethnic factors are not associated with ANCA positivity. After cessation of PTU, vasculitis did not develop during the 2 years of follow‐up despite positive ANCA.     

Development of Crohn’s disease following anti‐tumour necrosis factor therapy (etanercept)

Inhibition of tumour necrosis factor (TNF)‐alpha is effective in the treatment of rheumatoid arthritis and other inflammatory rheumatic diseases. Anti‐TNF antibodies such as infliximab, etanercept and adalimumab are commonly used. There are structural and functional differences among these agents. We describe development of Crohn’s disease in a patient with ankylosing spondylitis receiving anti‐TNF therapy. This case suggests that the appearance of gastrointestinal symptoms in patients on anti‐TNF therapy must be evaluated to find out whether this is a new onset or an exacerbation of underlying inflammatory bowel disease.     

Clinical and serological characteristics of seronegative primary Sjögren’s syndrome: a comparative study

Clinical Rheumatology - This study compared the clinical and serological characteristics of seronegative and seropositive primary Sjögren syndrome (pSS) and examined whether current...     

Diagnostic performance of minor salivary gland biopsy,serological and clinical data in Sj?gren’s syndrome: a retrospective analysis

The aim of this study was to investigate the performance of minor salivary gland biopsy (MSGB), serological and clinical data in diagnosis of primary Sjögren’s syndrome (pSS). Retrospective review of 216 patients who underwent minor labial salivary gland biopsy in last 5 years was performed. Results of the patients with diagnosis of pSS were compared with the patients failing to fill the classification criteria of pSS. Two groups did not differ significantly in terms of clinical symptoms and signs except presence of Raynaud’s phenomenon. Specificity and positive likelihood ratio of clinical signs in diagnosis of pSS were quiet low. A total of 78.7% of pSS patients had a focus score ≥1 (Chiscolm’s score III/IV) while all of the non-SS patients had a focus score <1 (P < 0.001). MSGB has the best predictive value with highest sensitivity and specificity for pSS diagnosis. Serological markers have higher predictive values compared to clinical symptoms and signs. Presence of Raynaud’s phenomenon, lymphopenia and/or hypergammaglobulinemia strengthens the probability of pSS in a patient with sicca symptoms.     

CTLA‐4 gene polymorphism of exon 1(+49 A/G) in Turkish systemic lupus erythematosus patients

Cytotoxic T lymphocyte‐associated antigen‐4 is a cell‐surface molecule providing a negative signal for T cell activation. CTLA‐4 gene polymorphisms are known to be related with genetic susceptibility to various autoimmune diseases, including systemic lupus erythematosus (SLE). However, the effects of this polymorphism on clinical features of SLE have not been defined. We analysed the CTLA‐4 gene +49 A/G polymorphisms in patients with SLE by using polymerase chain reaction–restriction fragment length polymorphism (PCR–RFLP) and investigated the effect of polymorphisms on clinical outcomes. Blood was collected from 47 unrelated Turkish SLE patients, all fulfilling the American College of Rheumatology criteria for SLE, and 100 ethnically matched healthy volunteers. The AA genotype was a predominant genotype in the Turkish population and genotype frequencies of CTLA‐4 AA were significantly higher in SLE patients (70%) than healthy controls (47%) (P = 0.015). There was a statistically significant difference in the AA genotype [odds ratio (OR): 2.66, confidence interval (CI) 95%: 1.27–5.56, P = 0.014] distribution among patients and controls. There was also an increase in A allele frequency in SLE and controls, but the difference was not statistically significant (81% vs. 70%, P = 0.068, OR = 1.8, CI 95%: 0.99–3.28). Interestingly, mean age and mean age of onset disease was higher in AA homozygote SLE patients compared to non‐AA (39.2 ± 11.5 vs. 31.6 ± 10.6, P = 0.044; 32.38 vs. 24.31, P = 0.046, respectively). There was no association between genotype and the other clinical features of SLE. Our results suggested that CTLA‐4 +49 AA genotype might be a risk factor for the development of SLE in Turkish population and G allele might be involved in early development of SLE. No association with clinical features was found for polymorphism of the promoter region in CTLA‐4 +49.     

Survival analysis of patients with Sjögren’s syndrome in Turkey: a tertiary hospital-based study

Clinical Rheumatology - This study was conducted to determine long-term survival rates and the factors associated with mortality in Turkish primary Sjögren syndrome (pSS) patients. All...