Low-dose rituximab is efficacious in refractory idiopathic inflammatory myopathies.

OBJECTIVES: Rituximab (RTX) use early in the course of refractory idiopathic inflammatory myopathy (IIM) is not well studied. This study sought to determine the short-term efficacy of RTX in a registry-based cohort of refractory IIM. METHODS: Registry-based observational data about IIM patients receiving RTX between 2018 and 2021 were included. Total improvement score was calculated from the core set measures as per International Myositis Assessment and Clinical Studies group (IMACS) at baseline, 6 months and 12 months of follow-up. RESULTS: Forty-two patients (F:M, 29:13), with a mean (s.d.) age of 39.5 (11.5) years were studied. Majority of patients received RTX for refractory myositis, after a median (interquartile range) duration of 8 (4,18) months. Twenty-eight received RTX at a dosage of 1 g × two doses, while 14 received 500 mg × two doses with an interval of 15 days. At 6 months and 12 months post-RTX, the improvement was recorded in manual muscle testing (MMT-8) scores, physician global assessment (PGA), patient global assessment (PtGA) and median steroid dosage as compared with the baseline (P < 0.01 for all). A mean (s.d.) improvement of 44.5 (16) and 48.7 (19.2) in total improvement score was recorded at 6 and 12 months, respectively. The change in MMT-8, PGA and PtGA scores from baseline between the two dosage regimens of RTX were comparable at 6 and 12 months. Severe lower respiratory tract infections requiring hospitalization occurred in three patients of the cohort. CONCLUSION: RTX improved IMACS core set measures and had steroid sparing efficacy at 6 and 12 months in patients with IIM in this registry-based study. Rituximab as an induction regimen of two doses of 500 mg can be as efficacious as 1 g at 6 months and 12 months of follow-up.

Risk factors associated with COVID-19 in systemic lupus erythematosus: Results from a longitudinal prospective cohort.

INTRODUCTION: Patients with SLE (systemic lupus erythematosus) have a higher risk of infection due to dysregulated immune system as well as long-term use of immunosuppressants (IS). This could influence the risk of COVID-19 and its outcome. METHODS: We conducted a longitudinal prospective study across 15 rheumatology centres during the first wave of the pandemic to understand the risk factors contributing to COVID-19 in SLE patients. During the 6 months follow-up, those who tested positive for COVID-19, their clinical course and outcome information were recorded. RESULTS: Through the study period (April-December 2020), 36/1379 lupus patients (2.9%) developed COVID-19. On analysing the COVID-19 positive versus negative cohort during the study period, male gender (adjusted RR 3.72, 95% C.I. 1.85,7.51) and diabetes (adjusted RR 2.94, 95% C.I. 1.28, 6.79) emerged as the strongest risk factors for COVID-19, in the adjusted analysis. There was no significant influence of organ involvement, hydroxychloroquine, glucocorticoid dosage (prednisolone< 7.5 mg or ≥ 7.5 mg/day) or IS on the risk of COVID-19. There was only one death (1/36) among the lupus patients due to COVID-19. CONCLUSION: Traditional risk factors rather than lupus disease process or IS influenced the risk of COVID-19 in our cohort.

Neuropsychiatric manifestations are associated with increased mortality in Indian patients with lupus: A single centre retrospective observational study.

OBJECTIVE: To study the prevalence of different NPSLE manifestations in our cohort and to compare clinical and immunological features and outcomes including mortality of patients with NPSLE and SLE controls without NP involvement. METHODS: This was a retrospective study in a tertiary care referral centre. All patients of SLE seen in the last 10 years and fulfilling the SLICC criteria with neuropsychiatric manifestations as per the ACR definitions were included. Patients of SLE without NP involvement were sequentially assigned as controls in a ratio of 1:2. RESULTS: Of the 769 patients diagnosed with SLE from Jan 2011 to December 2020, 128 (16.6%) had NPSLE manifestations as per the ACR definitions. The commonest NPSLE manifestation was seizures (6.5%) followed by cerebrovascular accident (3.9%). NPSLE manifestation occurred at the first presentation of SLE in 99/128 (77.3%) patients and 58 (45.3%) patients had more than one NPSLE manifestation. Lupus anticoagulant and anticardiolipin antibody were tested in 120 patients and were positive in 16 (13.3%) and 12 (10%), respectively. No difference was found in anti-ribosomal p, lupus anticoagulant and anticardiolipin antibodies between the cases and controls. Twenty-one (16.4%) deaths occurred in patients with NPSLE (median follow-up of 40 months) as compared to 13 (5%) in controls (median follow-up of 32 months) (p = <0.001). The cumulative survival of patients with NPSLE was lower as compared to controls (p < 0.001). Relapse of NPSLE was seen in 11(8.6%) patients and was associated with mortality (p = 0.017). CONCLUSIONS: Seizures and cerebrovascular accidents are the commonest NPSLE syndromes in our patients. The presence of NPSLE was associated with high mortality in Indian patients with lupus.

Does hydroxychloroquine improve patient reported outcomes in patients with lupus?

BACKGROUND: Hydroxychloroquine (HCQ) use is associated with less disease activity, flares, damage and improved survival in Systemic Lupus Erythematosus (SLE). However, its effect on patient reported health outcomes (PROs) such as quality of life (QOL) is not known. METHODS: International data from Study on Outcomes of Lupus (SOUL) from 2,161 SLE patients were compared by HCQ use. Disease activity and damage were assessed using SELENA-SLEDAI and SLICC-ACR/SDI. QOL was evaluated using LupusPRO and Lupus Impact Tracker (LIT). Linear regression analyses were performed with LupusPRO summary scores health related HRQOL, non-health related NHRQOL and LIT as dependent and HCQ use as independent variable. Analyses were undertaken to test mediation of effects of HCQ use on QOL through disease activity. RESULTS: Mean age was 40.5 ± 12.8 years, 93% were women. Sixty-three (1363/2161) percent were on HCQ. On univariate analysis, HCQ use was associated with (a) better QOL (LupusPRO-HRQOL: ß 6.19, 95% CI 4.15, 8.24, P ≤ 0.001, LupusPRO NHRQOL: ß 5.83, 95% CI 4.02, 7.64, P ≤ 0.001) and less impact on daily life (LIT: ß -9.37, 95% CI -12.24, -6.50, P ≤ 0.001). On multivariate and mediational analyses, the effects of HCQ on QOL were indirectly and completely mediated through disease activity. CONCLUSIONS: HCQ use in SLE is associated with better patient reported health outcomes (LupusPRO-HRQOL and NHRQOL and impact on daily life), and the effects are mediated through disease activity. This information can facilitate patients and physician's communication with decision-making regarding the use of HCQ for SLE management.

Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa.

The 1990 American College of Rheumatology (ACR) criteria for the classification of polyarteritis nodosa (PAN) have many pitfalls and performed poorly when used for diagnostic purposes. Recently, a provisional seven-item diagnostic criteria for PAN was proposed. To validate the provisional seven-item diagnostic criteria for PAN in a cohort of PAN patients from a tertiary care centre in India. Clinical details of patients diagnosed as PAN as per the European Medicines Agency algorithm between 2005 and 2020 were collected retrospectively. Age and sex-matched anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) patients were included in the non-PAN group. Patients with a deficiency of adenosine deaminase 2 (DADA2) were included as a separate group. The sensitivity, specificity, positive and negative predictive values (PPV and NPV) for ACR criteria, the Ministry of Health, Labour and Welfare (MHLW) in Japan diagnostic criteria and the seven-item diagnostic criteria were calculated. Thirty-seven PAN, 14 DADA2 and 37 AAV patients were included in the analysis. The sensitivity, specificity, PPV and NPV of the seven-item criteria were 83.7%, 96.8%, 97.3% and 81.1% respectively. For the ACR criteria, sensitivity was 82.9% and specificity was 79.5%. The sensitivity, specificity for MHLW criteria were 77.3% and 90% respectively. The sensitivity and specificity of seven-item criteria for DADA2 patients were 58.8% and 88.2% respectively. There was very poor agreement between the ACR criteria and the seven-item and MHLW criteria and fair agreement between seven-item and MHLW criteria (κ = 0.279). The provisional seven-item criteria for PAN performed well with high specificity and PPV.

Maternal and fetal outcomes of lupus pregnancies: A collective effort by Karnataka Rheumatologists.

INTRODUCTION: Identifying factors predicting adverse pregnancy outcomes involving systemic lupus erythematosus (SLE) is a research priority. The aims of this study were to investigate (a) the maternal and fetal outcomes of pregnant lupus patients and the factors associated with adverse pregnancy outcomes, and (b) the effect of pregnancy on lupus disease activity of these patients. METHODS: This was an ambi-directional study collecting information from five multi-specialist referral centres across the state of Karnataka, India over 5 years (2013-2018). Clinical details of pregnancies and outcomes that were temporally associated with lupus disease were recorded using a structured pro forma. The Safety of Estrogen in SLE National Assessment-SLE Disease Activity Index (SELENA-SLEDAI) was used to assess lupus activity during the 6 months prior to pregnancy and the intra- and post-partum periods. Modifications suggested in the SLE Pregnancy Disease Activity Index were considered while scoring. RESULTS: A total of 121 pregnancies in 80 SLE patients with a mean age of 27.1 (±4.5) years and with a mean disease duration of 4.6 (±4.1) years were reviewed. Largely patients were in clinical remission (109/121; 90.1%). Antiphospholipid antibody positivity was seen in 45/121 (37.2%) patients. A history of lupus nephritis was noted in 29/121 (24%) patients. Maternal complications (32%) were mainly due to hypertensive disorders of pregnancy (HDP; 19/121; 15.7%). Adverse fetal outcomes (58%) were mainly in the form of spontaneous first-trimester abortions (21/121; 16%), stillbirth (14/121; 11.6%) and prematurity (24/121; 20%). HDP is strongly associated with stillbirth and prematurity and is independent of active lupus. Disease activity was associated with a three-fold increased risk of adverse fetal outcome in univariate analysis. The risk of major flare during pregnancy is low (4.1%) when conception occurs during stable disease. Hydroxychloroquine (HCQ) use was associated with reduced risk of flare (p = 0.001) in patients in remission at the time of conception. CONCLUSIONS: The risk of major flare during pregnancy is low when conception happens during stable disease. HCQ use was associated with reduced risk of flare in patients in remission at the time of conception. HDP was strongly associated with stillbirth and prematurity and are independent of active lupus in our cohort.

Pulmonary and Ear, Nose and Throat (ENT) Involvement in ANCA-Associated Vasculitis at Diagnosis-Experience from a Tertiary Care Centre in North India.

BACKGROUND AND AIMS: There is paucity of data on pulmonary and ENT involvement in ANCA associatd vasculitis from India. We aimed to review the pattern of lung and upper respiratory tract involvement in patients with AAV diagnosed at our centre. METHODS: A retrospective review of all AAV patients between January 2007 and June 2014 was done. A complete clinical evaluation for Pulmonary and ENT involvement was done. Advanced investigations including computed tomography (CT) bronchoscopy and nasal endoscopy were done when indicated. Proportion of involvement was noted and different variables among patient groups were compared. RESULTS: 92 patients (median age 42 years; 60% female) of AAV were included. Clinical and/or radiological evidence of lung involvement was seen in 70 (76.1%) patients. Diffuse alveolar haemorrhage was present in 6 (60%) patients with MPA and 7 (10.1%) patients with GPA (p=0.002). ENT involvement was present in 55 (59.8%) patients and was more in GPA (p=0.000). Absence of renal involvement [p=0.047] and absence of GI involvement [p=0.012] were associated with ENT involvement in GPA. CONCLUSION: Pulmonary involvement was common in GPA, MPA and CSS, ENT involvement was almost characteristic of GPA. DAH was common in MPA. Population based and multicentre studies are needed to assess the true burden of organ involvement in AAV in the Indian population.

Giant cell arteritis in India: Report from a tertiary care center along with total published experience from India.

OBJECTIVES: Giant cell arteritis (GCA) is a granulomatous large vessel vasculitis with very scarce data from India. The purpose of this study was to present a comprehensive data of all published Indian cases along with our experience from North India. MATERIALS AND METHODS: This was a retrospective study of all patients diagnosed to be having GCA according to the American College of Rheumatology criteria at a large tertiary care hospital. The demographic data, clinical, investigations, treatment details, and outcomes were noted. Details of all case series and case reports published from India were pooled along with our experience in order to generate a cumulative data of all cases from India. This was then compared with several large published case series from South America, Europe, and Asia. RESULTS: A total of 72 patients (17 patients in the present series and another 55 patients from other Indian case series and case reports) were identified. The findings of our study are similar to the studies published from other parts of the world, except for the onset of the disease a decade earlier, a male predilection, a lower temporal artery biopsy positivity, and a higher incidence of ophthalmic complications. CONCLUSIONS: Indian patients with GCA have an earlier age of onset, male preponderance, and higher ophthalmic complications.

Occurrence and outcome of COVID-19 in AIRD patients on concomitant treatment with Tofacitinib- results from KRA COVID COHORT (KRACC) subset.

INTRODUCTION: We assessed the risk factors and outcome of COVID-19 in patients with autoimmune rheumatic diseases(AIRD) who contracted infection while on background treatment with tofacitinib. METHODS: This is a non-interventional, cross-sectional, questionnaire based telephonic study which included consecutive AIRD patients on tofacitinib co-treatment. Data related to the AIRD subset, disease modifying anti rheumatic drugs(DMARDs) including glucocorticoids and comorbidities, was collected from 7 rheumatology centers across Karnataka during the second wave of COVID-19 pandemic. The information about COVID-19 occurrence and COVID-19 vaccination was recorded. RESULTS: During the study period (Jun-July 2021), 335 AIRD patients (80.6% female) on treatment with tofacitinib were included. The mean duration of tofacitinib use was 3.4+/-3.1months. Thirty-six(10.75%) patients developed COVID-19. Diabetes mellitus (p = 0.04 (OR 2.60 (1.13-5.99)) was identified as a risk factor for COVID-19 in our cohort. Almost half of our cohort was COVID-19 vaccinated with at least one dose, with resultant decline in incidence of COVID-19(OR 0.15 (0.06-0.39) among the vaccinated. Recovery amongst COVID-19 infection group was 91.2%. CONCLUSIONS: The subset of AIRD patients who were on treatment with tofacitinib were found to have a higher rate of COVID-19 infection as compared to our KRACC cohort. Pre-existing comorbidity of diabetes mellitus was the significant risk factor in our cohort. This subset of the KRACC cohort shows RA patients had a lesser infection and PsA patients had a higher infection.

Long Term Outcomes in Idiopathic Inflammatory Myositis: An Observational Epidemiologic Study over 15 Years.

Background: We report a longitudinal observational cohort of idiopathic inflammatory myositis (IIM) focusing on the long-term clinical outcome and associated parameters. Methods: IIM patients were classified as per Bohan and Peter criteria. In those with ≥ 24 months of follow-up; the treatment response, functional outcomes, and damage at last follow-up were recorded. Complete clinical response and clinical remission as defined by Oddis et al., was used to define outcomes at last follow-up. Results: The cohort consists of 175 patients, mean age 40.9 (+12.6) years, M:F 1:3.3; and the major subsets were dermatomyositis (44.6%), overlap myositis (25.7%), antisynthetase syndrome (6.3%), polymyositis (14.3%), and juvenile DM/OM (8.6%). Ninety-four patients have followed up for 24 months or more, with the median (IQR) of 65(35,100.7) months. Of them, 74.1% and 11.8% had complete and partial clinical responses respectively at the last follow-up. In our cohort 40.2% were off-steroids and 13.8% were in clinical remission at the last follow-up. Complete clinical response was associated with better functional outcomes and lesser damage as determined by HAQ-DI of 0[OR10.9; 95%CI (3.3,160)], MRS [OR 3.2; 95%CI (1.4,7.3)] and lesser MDI [OR 1.7; 95% CI (1.1,2.7)] respectively as compared to partial response (unadjusted analysis). Baseline parameters and IIM subsets did not significantly influence the functional outcome and damage. The mortality rate in our cohort is 24/175 (13.7%), the disease-specific mortality rate being 9.1%. Large majority of deaths were early, associated with active disease. Conclusion: We report good long-term outcomes in all major myositis subsets. Partial clinical response to treatment is associated with worse functional outcomes and damage accrual. Death occurs early in association with active disease.

A prospective longitudinal study evaluating the influence of immunosuppressives and other factors on COVID-19 in autoimmune rheumatic diseases.

BACKGROUND: We conducted this study to identify the influence of prolonged use of hydroxychloroquine (HCQ), glucocorticoids and other immunosuppressants (IS) on occurrence and outcome of COVID-19 in patients with autoimmune rheumatic diseases (AIRDs). METHODS: This was a prospective, multicenter, non-interventional longitudinal study across 15 specialist rheumatology centers. Consecutive AIRD patients on treatment with immunosuppressants were recruited and followed up longitudinally to assess parameters contributing to development of COVID-19 and its outcome. RESULTS: COVID-19 occurred in 314 (3.45%) of 9212 AIRD patients during a median follow up of 177 (IQR 129, 219) days. Long term HCQ use had no major impact on the occurrence or the outcome of COVID-19. Glucocorticoids in moderate dose (7.5-20 mg/day) conferred higher risk (RR = 1.72) of infection. Among the IS, Mycophenolate mofetil (MMF), Cyclophosphamide (CYC) and Rituximab (RTX) use was higher in patients with COVID 19. However, the conventional risk factors such as male sex (RR = 1.51), coexistent diabetes mellitus (RR = 1.64), pre-existing lung disease (RR = 2.01) and smoking (RR = 3.32) were the major contributing risk factors for COVID-19. Thirteen patients (4.14%) died, the strongest risk factor being pre-existing lung disease (RR = 6.36, p = 0.01). Incidence (17.5 vs 5.3 per 1 lakh (Karnataka) and 25.3 vs 7.9 per 1 lakh (Kerala)) and case fatality (4.1% vs 1.3% (Karnataka) and 4.3% vs 0.4% (Kerala)) rate of COVID-19 was significantly higher (p < 0.001) compared to the general population of the corresponding geographic region. CONCLUSIONS: Immunosuppressants have a differential impact on the risk of COVID-19 occurrence in AIRD patients. Older age, males, smokers, hypertensive, diabetic and underlying lung disease contributed to higher risk. The incidence rate and the case fatality rate in AIRD patients is much higher than that in the general population.

The master of masquerade: A case series of unusual presentations of cutaneous tuberculosis in the immunocompromised host.

Cutaneous tuberculosis is known for its varied presentations, especially in the setting of immunosuppression. Clinical manifestations can be modified by the site of involvement as well as the type of cutaneous tuberculosis in a particular patient. Although cutaneous tuberculosis only accounts for a small percentage (1-2%) of extrapulmonary cases, it is encountered fairly frequently in dermatology practice in an endemic country like India. These cases are being presented to highlight unusual presentations of well-known forms of cutaneous TB (TB gumma and erythema induratum of Bazin) and rare morphologies (panniculitis) in immunosuppressed individuals, thereby emphasizing the need for the dermatologist in a TB-endemic country to be constantly vigilant.

Expanding spectrum of DADA2: a review of phenotypes, genetics, pathogenesis and treatment.

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic disease caused by biallelic mutations in ADA2 gene (previously CECR1). The aim of this review was to describe the clinical phenotypes, genetics, pathogenesis and treatment of DADA2. ADA2 is highly expressed on myeloid cells and deficiency leads to polarisation of macrophages to an M1 inflammatory type and activation of neutrophils. The pathogenesis of immunological and haematological manifestations is less clear. The spectrum of clinical presentations varies widely from asymptomatic individual to severe vasculitis, several autoinflammatory, immunological and haematological manifestations. Initially considered a childhood disease, the first presentation is now being reported well into adulthood. Vasculitis closely resembles polyarteritis nodosa. Livedoid reticularis/racemosa like skin rash and central nervous system involvement in the form of ischemic or haemorrhagic stroke are dominant manifestations. Immunological manifestations include hypogammaglobulinemia and recurrent infections. Lymphopenia is the most common haematological manifestation; pure red cell aplasia and bone marrow failure has been reported in severe cases. The disease is extremely heterogeneous with variable severity noted in patients with the same mutation and even within family members. Tumour necrosis factor inhibitors are currently the treatment of choice for vasculitic and inflammatory manifestations and also prevent strokes. Haematopoietic stem cell transplantation is a curative option for severe haematological manifestations like pure red cell aplasia, bone marrow failure and immunodeficiency. Further research is required to understand pathogenesis and all clinical aspects of this disease to enable early diagnosis and prompt treatment. Key Points • Deficiency of adenosine deaminase 2 (DADA2) is a monogenic disease caused by biallelic mutations in ADA2 gene. • The clinical features include vasculitis resembling polyarteritis nodosa, autoinflammation, haematological manifestations and immunodeficiency. • The severity varies widely from mild to fatal even in patients within a family and with the same mutation. • The treatment of choice for inflammatory and vasculitic disease is tumour necrosis factor α blockers. Bone marrow transplant may be considered for severe haematological disease.

Fatigue and its correlates in Indian patients with systemic lupus erythematosus.

Fatigue is a disabling yet poorly understood symptom in patients with systemic lupus erythematosus (SLE). Many variables influence fatigue including physical function, pain, fibromyalgia and psychiatric comorbidity. Literature is conflicted on its association with disease activity. To assess fatigue and its correlates among patients with SLE. Existent data from validation of Hindi LupusPRO study were utilized for this study. SLE patients were enrolled and underwent assessments of disease activity and damage (SELENA-SLEDAI and SLICC/ACR Damage Index (SDI)). Depression (Patient Health Questionnaire-9 (PHQ-9)), anxiety (Generalized Anxiety Disorder-7 (GAD-7)), fatigue (Fatigue Severity Scale (FSS)) and fibromyalgia were evaluated. Physical function and bodily pain were assessed using Short Form 36 (SF-36). Statistical analysis was done using the STATA version 14. A total of 140 females and 4 males with SLE with a mean age of 32.48 (SD 7.26) years were recruited. Median duration of illness was 3 years (IQR-4). Fatigue (FSS ≥ 4) was present in 74 (51.39%) participants. Prevalence of depression (PHQ-9 ≥ 10) and anxiety (GAD-7 ≥ 10) were 25% and 22.9%, respectively. Concomitant fibromyalgia was present in 8 participants (5.56%). Higher fatigue scores correlated with greater disease activity, shorter disease duration, worse physical function, bodily pain, depression and anxiety. On stepwise regression, disease duration, disease activity, bodily pain, depression and anxiety were independent predictors of fatigue (R2 = 0.35, p < 0.001). Fatigue is seen in over half of Indian patients with SLE and is independently associated with disease activity, disease duration, pain and psychiatric comorbidity. Multidisciplinary approach is indicated to address fatigue in SLE. Key Points • Fatigue is common in patients in Indian patients with SLE • Higher disease activity, shorter duration of disease, pain and psychiatric comorbidity were associated with fatigue.

Sexual functioning and its correlates in premenopausal married Indian women with systemic lupus erythematosus.

BACKGROUND: This study aimed to evaluate sexual functioning and its association with disease activity, damage, marital satisfaction, fatigue and psychiatric comorbidity in married women with systemic lupus erythematosus (SLE). METHODS: One hundred and twelve premenopausal married women with SLE were included in the study. Disease activity and damage were assessed using Safety of Estrogens in Lupus Erythematosus National Assessment Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) and Systemic Lupus International Collaborating Clinics/ American College of Rheumatology Damage Index (SDI) respectively. Female sexual function index (FSFI) and couple satisfaction index (CSI) were used to evaluate sexual function and marital satisfaction respectively. Depression, anxiety and fatigue were assessed using Patient Health Questionnaire 9 (PHQ9), Generalized Anxiety Disorder 7 (GAD7) and fatigue severity scale (FSS) respectively. RESULTS: The mean age of the study group was 34.0 (SD 6.8) years. Mean SELENA-SLEDAI was 3.67 (SD 4.2) and mean SDI was 0.25 (SD 0.62). Median steroid dose at the time of evaluation was 7.5 mg/d of prednisolone. Based on FSFI total score, impaired sexual functioning was found in 60.7%. However, when the cut-off of different domains was considered, more than 90% of the participants reported problems in desire, arousal and lubrication. The mean CSI score was 130.39 ± 26.17. Eighteen patients (16.1%) had CSI lower than the cut-off score (104.5) suggestive of marital distress. On univariate analysis sexual function showed a correlation with marital satisfaction (r = .34, P < .001), dose of steroids(r = -.26, P = .008), disease activity (r = -.21, P = .027), depression (r = -.19, P = .039) and anxiety (r = -.201, P = .034). CONCLUSION: Sexual dysfunction is highly prevalent in premenopausal married females with SLE. Higher dose of steroids, disease activity, depression,anxiety and marital satisfaction were associated with poor sexual functioning in one or more domains.

Comparison of the 2017 EULAR/ACR criteria with Bohan and Peter criteria for the classification of idiopathic inflammatory myopathies.

Bohan and Peter is the oldest criteria for the classification of idiopathic inflammatory myopathies (IIM). Recently, 2017 EULAR/ACR criteria were introduced which were validated against a control group. The objective of this study was to assess the performance of the 2017 EULAR/ACR criteria in retrospective cohort of adult and juvenile idiopathic inflammatory myopathies and compare with Bohan and Peter criteria. This was a retrospective study of patients clinically diagnosed to have IIM in a tertiary care center in the last 10 years. Only patients with a minimum follow-up of 6 months and response to steroids and immunosuppression were included in the study. Performance of both the criteria in the cohort was assessed and compared with clinical diagnosis. Hundred and eleven patients (87 females) were included in the study. Eleven patients had juvenile onset. Ninety-three patients (83.8%) were classified as probable/definite myositis using the Bohan and Peter criteria. Eighty-nine (80.2%) patients were classified as having probable/definite inflammatory myositis using the new criteria. Agreement between the two criteria was weak in our cohort (κ-0.331). Complete details of muscle biopsy were available in 52 patients. In this subgroup, 96% were classified by Bohan and Peter and 80.8% by EULAR/ACR criteria. Bohan and Peter classified 73% and EULAR/ACR 82% of patients when biopsy was excluded (n = 111). Both criteria classified over 90% of the patients with dermatomyositis. Forty-two patients were clinically diagnosed as polymyositis, of these 32 patients had myositis overlap syndrome. Bohan and Peter classified 66.7% and EULAR/ACR classified 64.3% in this subset. Bohan and Peter criteria had high sensitivity in the presence of muscle biopsy compared with EULAR/ACR. The performance of the EULAR/ACR criteria was similar to Bohan and Peter in the absence of muscle biopsy. Both criteria had poor sensitivity in polymyositis.

Neurological Manifestations Do not Affect Cumulative Survival in Indian Patients with Antineutrophil Cytoplasmic Antibody Associated Vasculitis.

BACKGROUND: Neurological manifestations are an important cause of morbidity in antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). It is not clear whether or not they are indicative of a severe disease course with multiple organ involvement and shortened survival. AIMS AND OBJECTIVES: To characterize the neurological manifestations of AAV and analyze their relationship with other organ system and cumulative survival. METHODS: This was a retrospective single-center cohort study of AAV patients at a tertiary care hospital in North India. Data was collected from medical records regarding clinical history, neurological examination, Birmingham Vasculitis Activity Score (BVAS), serology, electrophysiology, imaging, and histopathological examination findings of patients. RESULTS: Ninety-two patients of systemic vasculitis were identified, 67 with granulomatosis with polyangiitis (GPA), 14 with microscopic polyangiitis, 8 with Churg-Strauss syndrome (CSS), and 3 with undifferentiated AAV. The median BVAS at presentation was 18.0 (interquartile range (IQR): 12.0). The median duration of follow-up was 31.3 months (IQR: 40.5). A total of 45.7% patients had neurological manifestations among which 23.8% presented with neurological complaints. Peripheral neuropathy was the most common manifestation noted in 23.9% of the patients. Among patients with GPA, 40.3% had neurological involvement (seen in 33.3% patients at presentation). Patients with nervous system disease were more likely to have associated musculoskeletal manifestations (P = 0.046) and less likely to have renal involvement (P = 0.017). The estimated cumulative survival of the subgroup with neurological involvement was 95.1 months from the time of diagnosis, which was not significantly different from the cohort without neurological involvement (113.8 months, P = 0.631). CONCLUSION: Neurological morbidity commonly accompanies systemic vasculitis. Nervous system disease does not affect the survival significantly in these patients.

Clinical features and long-term outcomes of 105 granulomatosis with polyangiitis patients: A single center experience from north India.

AIM: To describe the clinical features, treatment and long-term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA). METHODS: Clinical details, Birmingham Vasculitis Activity Score (BVAS-v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories. The findings were compared with large single center cohorts from USA, Germany and France. RESULTS: One hundred and five patients (median age 40 years; 60 females and 45 males) were diagnosed as having GPA. Median duration of follow up was 28 months. Upper respiratory tract, renal and lung involvement were seen in 76.19%, 51.43% and 67.62% of patients, respectively. Ocular, ear, central nervous system, peripheral nervous system, heart, gastrointestinal and skin involvement were seen in 40.95%, 18.1%, 26.67%, 11.43%, 5.71%, 12.38% and 29.52%, respectively. The median BVAS v3 score was 17. Localized, early systemic, generalized, severe and refractory disease were seen in three, 44, 38, 17 and one patient, respectively. Eighteen patients expired and the cause of death was disease activity in 11, disease activity plus sepsis in four and sepsis alone in three patients. Twenty-six patients experienced relapses. No hemorrhagic cystitis or bladder cancers were observed; however, one patient developed myelodysplastic syndrome during follow up. CONCLUSION: Indian patients are younger and have less arthritis, renal and peripheral nerve involvement as compared to the patients in Western cohorts.

Polyarteritis nodosa in north India: clinical manifestations and outcomes.

OBJECTIVE: There has been a significant decrease in the number of published reports of classical polyarteritis nodosa (PAN) in the post-Chapel Hill consensus conference (CHCC) nomenclature era with only two series published from Asia. We report a case series of PAN from north India. PATIENTS AND METHODS: A retrospective study of all patients diagnosed to have PAN according to American College of Rheumatology criteria/CHCC nomenclature. The details of clinical presentation, investigation findings, treatment details and outcomes were noted from the records. These findings between the hepatitis B positive and negative groups were compared. RESULTS: Twenty-seven patients (20 male, seven female) were diagnosed as having PAN, out of which seven (25.9%) were hepatitis B surface antigen positive. Nervous system involvement was most common with 24 patients (88.9%) having mononeuritis multiplex. Weight loss was present in 20 (74%), fever in 14 (51.9%), renal involvement in 16 (59.3%), cutaneous in nine (33.3%), peripheral gangrene in eight (29.6%), gastrointestinal (GI) involvement in eight (29.6%), testicular pain in 6/20 (30%) and cardiac involvement in four (14.8%). Twenty-three (85.2%) patients recovered, three died (11.1%) and one was lost to follow-up. Median follow-up duration was 37 (interquartile range 22.00-69.75) months. The cumulative survival was 114.16 months (95% CI: 98.27-129.95). There was no significant difference in five factor score (FFS) or revised FFS between those patients who died and those who survived (P = 0.248, 0.894, respectively). Hepatitis B-related PAN had a lower FFS compared to non-hepatitis B-related PAN (P = 0.039). No other significant differences were noted between the two groups. CONCLUSION: In comparison to classic PAN in other populations, classic PAN in north India is associated with higher neurological involvement and lower GI involvement.