Clinical Characteristics and Outcomes of Interstitial Lung Disease in Primary Sjögren's Syndrome: A Retrospective Cohort Study.

Objectives: To describe the characteristics of primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD) and to assess treatment response. Methods: All patients of pSS from 2010 to 2019 were retrospectively identified. Lung function tests, high resolution computed tomography (HRCT) findings, and treatment outcomes were analysed. Results: Out of 550 patients with pSS, ILD was detected in 33 patients (frequency of 6 %). The mean(±SD) age at the diagnosis of pSS was 50 (± 9.3) years. 28/33(84.8%) were females. ILD onset preceded pSS diagnosis in 2 (6%) patients, simultaneously diagnosed in 21 (63.6%) patients and developed after pSS onset in 10 (30.3%) patients. 5 patients (15.15 %) were asymptomatic for ILD. Non-specific interstitial pneumonia (NSIP) accounted for the most frequent ILD subtype, in 15 patients (45.5%). Mycophenolate mofetil (MMF) was the most frequently used steroid sparing agent, in 25 patients (75.7%). 7 patients were lost to follow up. Response was seen in 22 patients, whereas 3 patients were non responders. There was one mortality due to lower respiratory tract infection-related sepsis. Presence of sicca symptoms [91.5% vs 8.7% (p<0.001)], NSIP pattern of ILD [90% vs 10% (p = 0.002)], and absence of Raynaud's phenomenon [91.7% vs 8.3% (p<0.001)] were significantly associated with responder status when compared to non-responders. Conclusion: ILD in primary Sjögren's syndrome is not an uncommon entity, and immunosuppression with steroids along with steroid-sparing agents led to good clinical outcomes of ILD in a majority of the patients in our cohort.

Hydroxychloroquine-Induced Phospholipidosis - A Forgotten Complication of a Common Drug.

Hydroxychloroquine (HCQ) has immunomodulatory and immunosuppressive properties and is used in many rheumatological conditions like systemic lupus erythematosus, rheumatoid arthritis, and Sjogren's syndrome. It is usually a widely used and well-tolerated DMARD (Disease Modifying Anti Rheumatic Drugs). Its most feared toxicities include retinopathy and, rarely, cardiomyopathy. Among its other reported side effects is drug-induced phospholipidosis. Here, we report two cases of HCQ-induced phospholipidosis based on renal biopsy electron microscopy. HCQ-induced phospholipidosis, although uncommon, must be considered as one of the differentials in a patient with persistent proteinuria.

Advances in Takayasu arteritis: An Asia Pacific perspective.

Takayasu Arteritis (TA) is a rare form of chronic granulomatous large vessel vasculitis that is more common in Asia compared to other parts of the world. There have been several developments in the field of Takayasu arteritis in relation to genetics, classification, clinical features, imaging, disease activity assessment and management and much of these works have been done in the Asia Pacific region. We will be discussing selected few in the current review.

Update on pregnancy in Takayasu arteritis-A narrative review.

Takayasu arteritis (TA) is a chronic, idiopathic large-vessel vasculitis that affects women of reproductive age, and has significant maternal and fetal implications. Although there are contrasting data on the effect of TA on fertility, most studies have shown that fertility outcomes remain unaffected. The disease activity of TA usually either remains stable or decreases during pregnancy. The important fetomaternal complications are maternal hypertension, pre-eclampsia, prematurity, and intrauterine growth restriction. To reduce maternal and fetal morbidity, controlling the disease before conception is important. This review article discusses the various implications, challenges, and medical and endovascular management of TA during pregnancy.