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.     

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.     

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...     

Early postoperative rupture of Heartware outflow graft: A case report

We know that new generation left ventricular assist devices (LVAD), significantly reduce the mortality of patients in the treatment of advanced heart failure disease, compared to optimal medical therapy. Day by day, we treat more heart failure patients with LVADs. Patients that can be cured are on the rise. But this also causes us to struggle with more complications. In this article, we present a case of cardiac tamponade due to rupture that occurred in the outflow graft of HeartWare left ventricular assist device (HVAD), a complication encountered for the first time as far as we know.     

Lung involvement in patients with primary Sjögren’s syndrome: what are the predictors?

The aim of this study was to investigate the prevalence, predictors and radiological findings of primary Sjögren’s syndrome (pSS)-associated lung involvement. This retrospective cohort study included 123 patients with demographic, clinical, laboratory and radiological data who were diagnosed with pSS. Lung involvement was defined based on the presence of pulmonary signs/symptoms and/or impaired pulmonary function tests along with alterations in high-resolution computerized tomography (HRCT). Thirty patients (24.4%) had pulmonary signs/symptoms at the initial presentation and/or during the follow-up period. Based on the criteria, 14 patients (11.4%) were defined as having pSS with lung involvement. The smoking rate, male/female ratio and the mean ages were found to be higher in patients with lung involvement (P < 0.05). Positive IgM-rheumatoid factor (RF), anti-La and anti-Ro results, the presence of hypergammaglobulinemia and lymphopenia had high specificity despite the low sensitivity rates to detect pSS-associated lung disease. A significant difference was found in forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV₁) results between the patients with and without lung involvement. Impaired FEV₁ had high specificity and positive predictive value compared to impaired FVC, particularly in non-smoker patients. The most frequent HRCT finding was ground-glass attenuation (64.3%). Other common findings were bronchiectasis, reticular pattern and honeycombing. The lesions involved predominantly the lower lobes. In conclusion, the presence of hypergammaglobulinemia and lymphopenia, positivity for RF, anti-La and anti-Ro, and impaired (FVC) and/or FEV₁ values could be the predictive parameters with a high specificity despite the low sensitivity rates. Smoking history, male gender and age are also risk factors. These parameters may be helpful to distinguish pSS-associated lung involvement from lung disorders unrelated to pSS.