This case report describes a case of retinal vasculitis in a patient with Rhupus, which has not been reported earlier in the literature. A 39-year-old female patient with a history of Rhupus presented with sudden vision loss in her left eye. The patient was treated earlier, for her polyarthritis, with oral corticosteroids, hydroxychloroquine, and oral methotrexate, which were discontinued after 1 year of treatment. At presentation, fundus examination of the left eye revealed mild vitritis, perivenous sheathing, and hemorrhagic periphlebitis. The patient was administered three doses of pulse corticosteroid, resulting in a reduction in retinal vasculitis and macular edema. The patient was continued on oral corticosteroids and started on oral methotrexate and hydroxychloroquine. At 6-week follow-up, the patient's vision had improved to 6/9, and fundus examination revealed resolving phlebitis and retinal hemorrhages. This case also highlights the importance of prompt diagnosis and treatment of retinal vasculitis in patients with Rhupus.

Objective The aim of this study is to develop a machine learning (ML) model to accurately predict liver enzyme elevation in rheumatoid arthritis (RA) patients on treatment with methotrexate (MTX) using electronic health record (EHR) data from a real-world RA cohort. Methods Demographic, clinical, biochemical, and prescription information from 569 RA patients initiated on MTX were collected retrospectively. The primary outcome was the liver transaminase elevation above the upper limit of normal (40 IU/mL), following the initiation of MTX. The total dataset was randomly split into a training (80%) and test set (20%) and used to develop a random forest classifier model. The best model was selected after hyper-parameter tuning and fivefold cross-validation. Results A total of 104 (18.2%) patients developed elevated transaminase while on MTX therapy. The best-performing predictive model had an accuracy/F1 score of 0.87. The top 10 predictive features were then used to create a limited feature model that retained most of the predictive accuracy, with an accuracy/F1 score of 0.86. Baseline high-normal transaminase levels, and higher lymphocyte and neutrophil blood count proportions were the highest predictors of elevated transaminase levels after MTX therapy. Conclusion Our proof-of-concept study suggests the possibility of building a well-performing ML model to predict liver transaminase elevation in RA patients being treated with MTX. Similar ML models could be used to identify "high-risk" patients and target them for early stratification.

Background: Fibromyalgia is characterized by chronic widespread pain, which has been linked to neuroinflammation. Hematological indices, i.e., neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and mean platelet volume (MPV) have been shown to be effective markers in neurological diseases like depression. Aims: To study the association between fibromyalgia severity and the hematological indices (NLR, PLR, and MPV). Subjects and Methods: This was a hospital-based cross-sectional study of fibromyalgia patients satisfying the 2016 modification of the 2010/11 ACR criteria. Demographic and clinical characteristics were recorded along with fibromyalgia outcomes and hematological indices. Statistical analysis was done using descriptive statistics, ROC analysis using the Youden index, and Pearson and Spearman correlations. Results: A total of 266 patients were recruited. The (mean +/- S.D) NLR, MPV, and PLR were 1.92 +/- 1.26, 8.94 +/- 1.25, and 119.48 +/- 76.91, respectively. Patients with severe visual analog scale (VAS) pain scores had lower MPV (8.8 +/- 1.3) than those with mild/moderate pain (9.2 +/- 1.1, p = 0.016). MPV showed a mild negative correlation with the Fibromyalgia Impact Questionnaire-Revised (FIQR) score (R2 -0.31 p 0.004). MPV threshold of 8.95 was discriminated severely from mild/moderate VAS-pain score with a sensitivity of 52.3 % and specificity of 66.7%. Conclusions: MPV can possibly be considered as a biomarker for predicting pain severity in fibromyalgia. Given its inexpensive nature, MPV can be used as a cost-effective method to assess fibromyalgia severity in rural India.

Vogt–Koyanagi–Harada (VKH) disease presents as a bilateral granulomatous panuveitis with systemic manifestations. We report a 60-year-old female who presented with features suggestive of VKH and was treated with steroids and immunosuppression. Although thorough investigation ruled out tuberculosis (TB) at presentation, she developed neurotuberculosis 2 months after initiating treatment. The primary diagnosis of VKH was hence revisited. Since both VKH and TB uveitis can present as chronic granulomatous panuveitis, it is important to differentiate between them. Once started on immunosuppression, patients should be kept on close follow-up for early detection of development of infections or reactivation of latent TB.

Background: Fibromyalgia (FM) is a rheumatologic disease that advances with chronic pain and disability and represents one of the most common causes of chronic and diffuse pain in the population. Objective: An observation study was conducted to compare the role of an individually administered form of Affective Cognitive Behavioural Therapy (ACBT) with Jacobson's Progressive Muscular Relaxation (JPMR) in changing the scores of Pain and FM impact among patients with FM. Method: Patients receiving ACBT and JPMR were allocated to two groups by licensed clinical psychologists until each group had a sample of 10 each. Both groups received 6 individual sessions (once in every two weeks). A baseline assessment was done to assess the Pain and FM impact prior to the intervention. Post and follow up assessments were carried one week and one month after the intervention respectively. Post scores of both groups were compared with baseline scores. Results: The current study revealed that ACBT is associated with impressive improvements in Pain reduction and FM Impact among the patients with FM. Conclusion: Considering the effect of ACBT in reducing the Pain and FM Impact scores, ACBT shall be considered as an add-on psychological intervention along with the pharmacological management to yield better outcomes in FM patients. However, more research including randomized control trails are required to confirm its effectiveness.

Introduction. There is a scarcity of information available on clinical and laboratory features of adult-onset idiopathic autoimmune uveitis. Therefore, we conducted a single centre descriptive cross-sectional study. Patients and Methods. A chart review of all patients with idiopathic autoimmune uveitis with onset after 18 years of age who were referred to the rheumatology department between January 2017 and December 2018 was performed. Their clinical features, demographic features, and HLA-B genotypes were documented and described. Results. Out of 210 patients referred to rheumatology, 66 were found to have uveitis, and 16 of these had an adult-onset idiopathic autoimmune uveitis. Apart from a slight female preponderance (62.5%), our patients were characterized by a high proportion of panuveitis (4 out of 16, i.e., 25%). There was an increased frequency of occurrence of synechiae (5 out of 16, i.e., 31.3%), retinal vasculitis (4 out of 16, i.e., 25%), optic disc edema (3 out of 16, i.e., 18.8%), and cystoid macular edema (seen in 2 patients, i.e., 12.5%). These features correlated with the anatomical subtypes. Retinal vasculitis and optic disc edema present in three fourth of all panuveitis cases were the most prominent features. The odds of finding HLA-B∗35 in retinal vasculitis were 33 times higher than odds of finding it in idiopathic autoimmune uveitis patients not having retinal vasculitis (OR 33; 95% CI 1.6–698). Conclusion. Idiopathic autoimmune uveitis in our patients is characterized by a high frequency of panuveitis and retinal vasculitis, and complications with a probable association between HLA-B∗35 and retinal vasculitis.

Objectives: Sulfasalazine has been widely used in treatment of rheumatoid arthritis and spondyloarthritis. This study aims to assess persistence with sulfasalazine and also frequency and severity of adverse drug reactions (ADRs) encountered with this very well-established disease-modifying anti-rheumatoid drug. Materials and methods: This retrospective study was done in 1,114 patients from medicine and rheumatology outpatient departments of six centers across India. The inclusion criteria was patients taking sulfasalazine. Patients receiving sulfasalazine for rheumatoid arthritis or spondyloarthritis were selected and details on drugs used, duration of taking sulfasalazine, ADRs to sulfasalazine and whether sulfasalazine had to be stopped due to ADRs were analyzed. Results: Of the total of patients included in the study, 10.1% had ADRs with sulfasalazine, and stopped the drug. Gastritis, deranged liver enzymes, hepatitis, skin rashes were the most commonly encountered ADRs. Of the total number of patients recruited for the study, 11% were lost to follow-up, as most of the centers were army hospitals and the officers and staff were posted to other places. Sulfasalazine was taken for less than 1 month by 3.8% patients while 12.5% had taken the drug for less than 6 months. Of the study patients, 28.6% had taken the drug for 24 - 60 months and 4.6% (51 patients) had taken it for more than 5 years. It was found that ADRs were most commonly encountered within the first year of using the drug, and persistence was seen in patients on long-term therapy. Conclusion: Sulfasalazine is a safe option in chronic therapy of rheumatoid arthritis and spondyloarthritis. Although frequency of ADRs with patients taking sulfasalazine is minimal, it did necessitate the stoppage of drug. If not well tolerated, sulfasalazine would not have been continued for more than 12 months as evidenced from this study.

Melanoma differentiation-associated protein 5 (MDA5) antibody-positive dermatomyositis (DM) displays unique cutaneous and pathologic features. We describe two cases of myositis-associated rapidly progressive interstitial lung disease (RP-ILD). The patients were two women from Kerala, India. Both patients had anti-MDA5 antibody-positive myositis. Both patients presented with RP-ILD without any clinical features of myositis and succumbed to their illness despite aggressive medical treatment. Anti-MDA5-antibody-positive DM is characterised by amyopathic disease with rapidly progressive and fatal ILD.

Background: Fibromyalgia is a chronic pain syndrome characterized by widespread diffuse pain and multiple tender points along with sleep disturbances, fatigue, and psychological distress. Cytokines have been proposed to play an important role in the pathogenesis of fibromyalgia. Different studies, mainly done in the Western population, have shown dysregulated cytokines in fibromyalgia patients. This study was aimed to analyze the serum cytokine patterns of interleukin-1 receptor antagonist (IL-1Ra), interleukin-8 (IL-8), and interleukin-6 (IL-6) in Indian females with fibromyalgia and its correlation with the disease severity. Materials and Methods: This single-center cross-sectional study compared the serum cytokines levels (IL-6, IL-8, and IL-1Ra) of 21 females fulfilling 2016 modification of the 2010/11 American College of Rheumatology fibromyalgia criteria and without co-existent major depressive or inflammatory diseases; with 20 age- and sex-matched controls. The outcome measures used to measure disease activity were visual analog scale pain, fibromyalgia impact questionnaire-revised, brief pain inventory pain scores, and SF-36 health survey. Statistical tests used were the Kolmogorov–Smirnov test of normality, Mann–Whitney's test, Chi-square tests, Spearman rank order, and Benjamini–Hochberg correlation. Results: Serum IL1-Ra levels were significantly lower in fibromyalgia patients, median 226.90 pg/ml (interquartile range [IQR] 476.95) than the healthy controls, median 778.80 pg/ml (IQR 721) (P < 0.05). However, there was no statistical difference in the levels of IL-8 and IL-6 between patients and the control group. The analysis for the correlation between cytokine and outcome measures failed to show any statistically significant correlation. Conclusion: Fibromyalgia patients in our study had lower serum IL-1Ra level, whereas IL-8 and IL-6 levels were normal. When compared with western studies, these contrasting results suggest a heterogeneous cytokine profile of fibromyalgia patients in different ethnic groups.

Background In autoimmune inflammatory rheumatological diseases, routine cardiovascular risk assessment is becoming more important. As an increased cardiovascular disease (CVD) risk is recognized in patients with fibromyalgia (FM), a combination of traditional CVD risk assessment tool with Machine Learning (ML) predictive model could help to identify non-traditional CVD risk factors. Methods This study was a retrospective case–control study conducted at a quaternary care center in India. Female patients diagnosed with FM as per 2016 modified American College of Rheumatology 2010/2011 diagnostic criteria were enrolled; healthy age and gender-matched controls were obtained from Non-communicable disease Initiatives and Research at AMrita (NIRAM) study database. Firstly, FM cases and healthy controls were age-stratified into three categories of 18–39 years, 40–59 years, and ≥ 60 years. A 10 year and lifetime CVD risk was calculated in both cases and controls using the ASCVD calculator. Pearson chi-square test and Fisher's exact were used to compare the ASCVD risk scores of FM patients and controls across the age categories. Secondly, ML predictive models of CVD risk in FM patients were developed. A random forest algorithm was used to develop the predictive models with ASCVD 10 years and lifetime risk as target measures. Model predictive accuracy of the ML models was assessed by accuracy, f1-score, and Area Under 'receiver operating Curve' (AUC). From the final predictive models, we assessed risk factors that had the highest weightage for CVD risk in FM. Results A total of 139 FM cases and 1820 controls were enrolled in the study. FM patients in the age group 40–59 years had increased lifetime CVD risk compared to the control group (OR = 1.56, p = 0.043). However, CVD risk was not associated with FM disease severity and disease duration as per the conventional statistical analysis. ML model for 10-year ASCVD risk had an accuracy of 95% with an f1-score of 0.67 and AUC of 0.825. ML model for the lifetime ASCVD risk had an accuracy of 72% with an f1-score of 0.79 and AUC of 0.713. In addition to the traditional risk factors for CVD, FM disease severity parameters were important contributors in the ML predictive models. Conclusion FM patients of the 40–59 years age group had increased lifetime CVD risk in our study. Although FM disease severity was not associated with high CVD risk as per the conventional statistical analysis of the data, it was among the highest contributor to ML predictive model for CVD risk in FM patients. This also highlights that ML can potentially help to bridge the gap of non-linear risk factor identification.

Objectives: The objectives of the study were to study the demographic, clinical, serological, and imaging characteristics of connective tissue disease-associated interstitial lung disease (CTD-ILD) patients seen at rheumatology centers across India. Methods: Adult outpatients with CTD-ILD and interstitial pneumonia with autoimmune features (IPAF), who were seen at the 12 participating rheumatology centers at least once in the preceding 6 months, were recruited and information was retrieved from their medical records. Information on demographics, duration of symptoms, diagnosis, comorbidities, autoantibodies, lung imaging, pulmonary function testing (PFT), echocardiography (ECHO), treatment, immunization, and admissions for exacerbations was obtained. Results: A total of 620 patients (505 women) were recruited. Rheumatoid arthritis (RA) was the most common CTD associated with ILD followed by systemic sclerosis and mixed connective tissue disorder. In our cohort, 372 (60%) patients had not received a diagnosis of their rheumatic disease or the ILD before seeing their rheumatologist. High-resolution computed tomography of the chest was available for 551 (88.9%) of the patients, and 327 (59.3%) had a nonspecific interstitial pneumonitis (NSIP) pattern. PFT and ECHO data were available for 437 (70.5%) and 453 (73.1%) patients, respectively. The mean forced vital capacity was 61.89% ± 14.8% of the predicted. Pulmonary hypertension (PH) was detected in 162 (35.8%) patients. The most common immunosuppressive used for lung disease was mycophenolate mofetil (54.0%) followed by azathioprine (19.5%). Medical records of these patients showed that after a diagnosis of ILD, in 79 patients, methotrexate (MTX) was changed to an alternative drug, whereas in 11 patients, MTX was added by the rheumatologist after diagnosing the CTD-ILD. There were 28 patients with IPAF. Mycophenolate and rituximab were the common drugs used in IPAF patients. Only 36 (5.8%) of the 620 patients were vaccinated with influenza as well as both the conjugate and the polysaccharide pneumococcal vaccines. Seventy-four (12.25%) patients had a history of one or more admissions for acute exacerbations or complications of ILD. Conclusion: In this study, RA-ILD was the most common CTD and NSIP was the predominant ILD pattern on high-resolution computerized tomography. More than a third of the patients had PH. In the real-life scenario, we noticed hesitancy among the treating doctors in using MTX in RA-ILD. The number of patients with complete influenza and pneumococcal vaccination was low and this issue needs to be specifically addressed. Larger prospective registry-based data are needed to understand CTD-ILD and the unmet needs in the Indian context.

Fig. 4. Histopathology image: Skin biopsy, from papulopustular lesion over the upper back. Image showing ill-to-well-formed granulomas (hollow arrow) with giant cells, interstitial giant cells and histiocytes in the upper dermis (H and E, ×10). Fig. 3. Positron-emission tomography-magnetic resonance image showing FDG avid multiple mediastinal and intra-abdominal lymph nodes, FDG avid and non-avid T2 hyperintense lesions in the spleen (arrows noted A in image) and FDG non-avid T2 hyperintense lesions in the liver (arrows noted B in image).