Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study.

The current study aimed to characterize patients from a rheumatology referral center in north India, who satisfied the definition of interstitial pneumonia with autoimmune features (IPAF) as given by the American Thoracic Society and European Respiratory Society (ATS/ERS) consensus committee in 2015. Thirty-five adult patients aged 18 years and above, fulfilling the 2015 ATS/ERS criteria for IPAF were included in the study. The clinical and immunological profile, and radiologic findings on high-resolution computerized tomography thorax were noted. Antinuclear antibody (ANA) by indirect immunofluorescence at 1:320 titer and myositis-specific antibody (MSA) assays were performed. Non-parametric tests were used to compare variables between groups. The study cohort included predominantly female patients with a mean age of 50.6 ± 13 years and mean duration of disease of 38.8 ± 28.4 months. Majority of patients (49%) fulfilled the morphologic and serologic domains as per the IPAF consensus criteria and 31% patients had features in all three domains. Non-specific interstitial pneumonia was the most common pattern observed in 77% patients. Raynaud's phenomenon and inflammatory arthritis were the predominant autoimmune features. Pulmonary arterial hypertension was documented in 60% of patients on echocardiography. Positive ANA at 1:320 dilution was present in all 26 patients tested, whereas extractable nuclear antigen and MSA assays detected autoantibodies in 49% and 51% of patients respectively. IPAF predominantly affected females in the age group of 50 years and above, with varied autoimmune manifestations and autoantibody profile.

Safety of the ChAdOx1 nCoV-19 and the BBV152 vaccines in 724 patients with rheumatic diseases: a post-vaccination cross-sectional survey.

Patients with rheumatic and musculoskeletal (RMD) diseases may be at higher risks for COVID-19 infection. Data on the safety of the adenoviral vector-borne ChAdOx1 nCoV-19 and the heat-inactivated BBV152 Vaccines in this group are limited. 724 patients with RMD who had received at least one dose of either the ChAdOx1 or the BBV152 were audited to find out post-vaccination adverse effect (AE) or disease flares. The AE rates in patients with autoimmune rheumatic disease (AIRD) were compared with those with non-AIRD RMDs. The mean age of the cohort was 59.9 (± 10.43) years with a female (n = 581; 80.24%) majority. 523 (70.8%) had AIRD. The ChAdOx1 and the BBV152 vaccines were received by 624 (86.18%) and 77 (10.63%), respectively. 23 (3.17%) were unaware of which vaccine they had received. 238 (32.87%) of patients had at least one comorbidity. 436 (60.22%) participants [306 (59.64%) of those with AIRD and 130 (61.61%) with other RMDs] had at least one adverse effect (AE). Four patients reported flare of arthritis that resolved within 5 days. No patient had any severe AE or required hospitalization. All AEs were self-limiting. Both the ChAdOx1 and the BBV152 vaccines appear safe in RMDs. AEs do not differ between patients with AIRD or non-AIRD. This information can help negate vaccine hesitancy amongst all stakeholders.

Prevalence of liver fibrosis by Fibroscan in patients on long-term methotrexate therapy for rheumatoid arthritis.

INTRODUCTION: Data on the long-term use of methotrexate (MTX) causing liver fibrosis in patients with rheumatoid arthritis (RA) is sparse. Liver biopsy is the gold standard to assess fibrosis but is an invasive procedure. Transient elastography (TE) by Fibroscan is a noninvasive validated tool to detect and quantify liver fibrosis. The present study aimed to assess the prevalence of liver fibrosis by Fibroscan in patients with RA on long-term MTX therapy and its correlation with cumulative dose of MTX. METHODS: This cross-sectional study included adult patients (≥ 18 years age) of RA who had been on MTX for ≥ 3 years. The patients' records were reviewed, and the cumulative dose of MTX was calculated. Liver fibrosis was assessed by TE method, and the cutoff value of 7.1 kPa (kilopascal) was considered abnormal (liver fibrosis). Spearman's rank test was used to assess the correlation between the cumulative dose of MTX and Fibroscan score. RESULTS: Seventy-five patients were enrolled of which 69 were females (92%). The mean age was 47.2 ± 11.3 years. The mean body mass index and waist circumference were 24.8 ± 3.9 kg/m2 and 91.6 ± 9.9 cm, respectively. The median duration and cumulative dose of MTX were 336 weeks (interquartile range,144-912 weeks) and 6300 mg (interquartile range, 2400-22,000 mg), respectively. The mean liver stiffness was 5.22 ± 2.03 kPa. Twelve patients (16%) had Fibroscan score ≥ 7.1 kPa, of which 3 patients had severe liver stiffness (9.5 to 12.5 kPa) and one patient had liver stiffness in the range of cirrhosis (> 12.5 kPa). Fibroscan scores significantly correlated with cumulative dose of MTX (r= 0.30, p = 0.008). CONCLUSIONS: Long-term MTX therapy in RA was associated with increased liver stiffness on Fibroscan. Key Points • Fibroscan is a useful tool for monitoring MTX-induced liver fibrosis. • Liver fibrosis as evidenced by increased liver stiffness on Fibroscan is prevalent among patients on long-term MTX therapy for RA.

Prevalence of hydroxychloroquine retinopathy with long-term use in a cohort of Indian patients with rheumatic diseases.

The study aims to estimate the prevalence of hydroxychloroquine (HCQ) retinopathy in a cohort of Indian patients and analyse the associated factors. Adult patients with rheumatological disorders aged ≥ 18 years using HCQ for more than 5 years and/or having received a cumulative dose > 400 g were included. Demographic and clinical data were collected and all underwent ophthalmological tests which included Humphrey automated visual fields (AVF) and spectral domain optical coherence tomography (SD-OCT). The various clinical characteristics of the patients were compared. The study included 110 patients with a mean age of 43.5 ± 10.1 years and predominantly females. Eleven patients (10%) were diagnosed with definite HCQ retinopathy. The mean daily dose of HCQ (mg/kg of real body weight) was significantly different in the groups with and without retinopathy (5.7 ± 0.9 vs 5.1 ± 0.8, p = 0.04). Patients with retinopathy had significantly more colour vision abnormalities (odds of 16.9; confidence interval 4.1-69.1, p = 0.0001) and higher prevalence of both parafoveal and perifoveal thinning (p < 0.0001). Age, gender, duration of HCQ use, cumulative HCQ dose and body mass index were not found to be associated with retinopathy. Four out of 11 patients had abnormalities only on 30-2 protocol for AVF testing, two had abnormalities only on 10-2 protocol, whereas five patients had abnormalities on both protocols. SD-OCT abnormalities were present in all patients with retinopathy. Hydroxychloroquine retinopathy was prevalent in the study cohort and significantly associated with a higher daily dose of HCQ (mg/kg real body weight).

Aggressive eosinophilic granulomatosis with polyangiitis and transverse sinus thrombosis.

We report a case of a patient with eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) who succumbed to respiratory failure complicated by sepsis, pneumonitis and transverse sinus thrombosis. In the absence of antineutrophilic cytoplasmic antibodies positivity, a clinical diagnosis was made based on The American College of Rheumatology-1990 criteria. Glucocorticoids, cyclophosphamide, rituximab and intravenous immunoglobulin were administered at different stages. The patient recovered momentarily after each cycle of treatment, but his vasculitis progressed and he eventually died. Cardiac or renal involvement, which is more commonly associated with an adverse outcome, was not present.