Semi-quantitative thigh magnetic resonance imaging scores in assessing disease activity and determining long-term clinical outcome in idiopathic inflammatory myopathies: a causal mediation analysis.

OBJECTIVES: To evaluate the relationship of thigh MRI (t-MRI) with manual muscle testing-8 (MMT-8), muscle enzymes and autoantibodies. To determine the causal and mediating factors resulting in poor recovery of MMT-8 in inflammatory myositis (IIM). METHODS: This was a single-centre retrospective study in IIM patients. t-MRI was semi-quantitatively scored for muscle oedema, fascial oedema, muscle atrophy and fatty infiltration. Spearman correlation of t-MRI scores was done with muscle enzymes at baseline, and MMT-8 at baseline and on follow-up. Causal mediation analysis was performed with age, sex, symptom duration, autoantibodies, diabetes and BMI as independent variables, follow-up MMT-8 as dependent and t-MRI scores as mediating variables. RESULTS: Baseline evaluation was done on 59 and follow-up on 38 patients. Median follow-up of the cohort was 31 (10-57) months. Baseline MMT-8 negatively correlated with muscle oedema (r = -0755), fascial oedema (r = -0.443) and muscle atrophy (r = -0.343). Creatinine kinase (r = 0.422) and aspartate transaminase (r = 0.480) positively correlated with muscle oedema. Follow-up MMT-8 correlated negatively with baseline atrophy (r = -0.497) and fatty infiltration (r = -0.531). On follow-up, MMT-8 males had positive total effect (estimate (95%CI)) via atrophy [2.93 (0.44, 4.89)] and fatty infiltration [2.08 (0.54, 3.71)]. Antisynthetase antibody had a positive total effect via fatty infiltration [4.50 (0.37, 7.59)]. Age had a negative total effect via atrophy [-0.09 (0.19, -0.01)] and fatty infiltration [-0.07 (-0.15, -0.01)]. Disease duration had a negative total effect via fatty infiltration [-0.18 (-0.27, -0.02)]. CONCLUSION: Baseline fatty infiltration and muscle atrophy resulting from older age, female sex, longer disease duration and absent anti-synthetase antibodies, partly mediate muscle recovery in IIM.

Type I interferon gene expression signature as a marker to predict response to cyclophosphamide based treatment in proliferative lupus nephritis.

OBJECTIVES: To assess the longitudinal effect of cyclophosphamide (CYC) treatment on type-I interferon (IFN) signature in proliferative lupus nephritis (LN) and its role in predicting treatment response. METHODS: Fifty-four biopsy proven proliferative LN patients scheduled to receive high-dose (HD) or low-dose (LD) CYC were recruited and followed up for six months. At six months, patients were classified as clinical responders (CR) or non-responders (NR) to treatment, using the EULAR/EDTA criteria. An IFN-gene based score (IGS) was developed from the mean log-transformed gene expression of MX1, OAS1, IFIT1, OASL, IFIT4, LY6E, IRF7 at baseline, three and six months. Longitudinal changes of IGS within and between groups were assessed and ΔIGS, which is the difference in IGS between baseline and three months was calculated. Independent predictors of non-response were identified and an ROC analysis was performed to evaluate their utility to predict NR. RESULTS: There was a dynamic change in IGS within the HD, LD, CR, and NR groups. Compared to baseline, there was a significant decrease in IGS at three months in HD and LD groups (HD group: 2.01 to 1.14, p = .001; LD group = 2.01 to 0.81, p < .001), followed by a significant increase from three to six months in LD group (LD: 0.81 to 1.51, p = .03; HD: 1.14 to 1.54, p = .300). A decrease in IGS from baseline to three months was seen in both CR (2.13 to 0.79, p < .001) and NR groups (1.83 to 1.27, p = .046), and a significant increase from three to six months was observed only in the CR group (CR: 0.79 to 1.57, p = .006; NR: 1.27 to 1.46, p = 1). ΔIGS (baseline to three months) was higher in CR compared to NR group (-1.339 vs -0.563, p = .017). ROC analysis showed that the model comprising of 0.81 fold decrease in IGS from baseline to three months, endocapillary hypercellularity and interstitial inflammation on renal histopathology predicted non-response with a sensitivity of 83.3% and specificity of 71.4%. CONCLUSION: In proliferative LN, treated with HD or LD-CYC, combined model comprising of decrease in IGS score by 0.81 fold from baseline to three months, along with important histopathological features such as endocapillary hypercellularity and interstitial inflammation had better predictive capability for non-response.

Outcome of critically ill patients with systemic lupus erythematosus from a medical intensive care unit in Southern India.

OBJECTIVE: Systemic lupus erythematosus (SLE) has become the most prevalent autoimmune condition requiring admission in the intensive care units (ICU) in the last two decades. Here we analysed the clinical outcomes of SLE patients admitted to our ICU between 2011 and 2021, and studied the prognostic role of high-density lipoprotein (HDL) and procalcitonin in those enrolled after August 2019. METHODS: Systemic lupus erythematosus (ACR/SLICC 2012) were enrolled, 72 retrospectively and 30 prospectively. Data on indications for ICU admission, complications, infections, and disease activity were recorded. Outcome was mortality at 90 days (prospective) whereas in the retrospective analysis outcome was hospital discharge or death in hospital. Serum HDL and procalcitonin (PCT) was estimated in the prospectively enrolled 30 patients and compared with 30 non ICU-SLE patients. RESULTS: Indications for ICU admissions were respiratory causes in 78/102 (76.5%) patients; for haemodynamic monitoring and for invasive procedures in the remaining. Pneumonia was the primary reason for mechanical ventilation, followed by diffuse alveolar haemorrhage (DAH). Eighty-three (81.3%) patients died; infections (n = 54) and SLE related causes (n = 29). APACHE-II >16 (p = .026), lymphopenia (p = .021), infection (p = .002), creatinine >1.3 mg/dL (p = .023), and hypotension requiring vasopressor support (p = .006) emerged as significant predictors of non-survival on multivariable analysis. HDL (mg/dL) day 1 was significantly lower in SLE-ICU patients compared to non ICU-SLE (31.8 ± 14.3 vs 38.8 ± 11.4 mg/dl); p = .045. On day 1, PCT (ng/mL) in SLE-ICU was significantly higher when compared to non-ICU SLE; median (IQR): 0.53 (0.26-5.27) versus 0.13 (0.05-0.47), p < .001), respectively. It was also significantly higher on day 5 in SLE-ICU than non-ICU SLE (median (IQR): 4.18 (0.20-14.67) versus 0.10 (0.08-0.46), p = .004. CONCLUSION: The mortality of SLE patients admitted to the ICU in this study is high, and infections were the principal reason for death. Baseline low HDL and higher procalcitonin are potential biomarkers to identify critically ill SLE patients.

Kidney histopathology in predicting flares following drug withdrawal in proliferative lupus nephritis in clinical remission.

Residual renal histopathological activity at clinical remission in Proliferative Lupus Nephritis (PLN) can predict renal flare upon immunosuppression withdrawal. Data on the role of histological renal remission in predicting extra-renal flares is lacking. We assessed renal histopathology prior to drug withdrawal and the occurrence of renal and extra-renal flares over 52 weeks after drug withdrawal in PLN patients in long-term clinical remission. This is a subgroup analysis of a non-inferiority, open-label randomized (1:1) controlled trial. Patients with biopsy-proven Class III/IV LN in the past (biopsy 1), on immunosuppressants (IS) ≥ 3 years, in clinical remission for ≥ 1 year, on stable prednisolone dose (≤ 7.5 mg/day) plus a maintenance IS and hydroxychloroquine (HCQ) were subjected to a repeat renal biopsy (biopsy 2). Individuals with biopsy 2 having activity index (AI) < 4/24 were randomised to either prednisolone or IS withdrawal. Primary end-point was the proportion experiencing a flare [SELENA-SLEDAI flare index (SFI)] at week 52. Twenty-eight eligible patients underwent biopsy 2 and randomized to prednisolone (n = 15) and IS (n = 13) withdrawal. At biopsy 1, 12 (43%) had class III, 15 (53.5%) had class IV, and 1 (3.5%) had class III + V. At biopsy 2, PLN persisted in 4 (14.2%) while 18 (64.2%) were in histological remission (AI = 0) with 6 (21.4%) in class II. Following drug withdrawal, 9/28 (32%) had flares especially musculoskeletal (55.5%), mucocutaneous (44.4%), and renal (33.3%). Among the four persistent PLN patients, one of the two (50%) with AI = 1 had extra-renal flare while both the two with AI = 2 (100%) had renal and extra-renal flares. In those with histological remission (biopsy 2), 6/18 (66.6%) experienced extra-renal flare of whom one also had renal flare. Upon drug withdrawal, renal histopathology findings with any activity index can predict renal flare while histological remission is not enough to predict extra-renal flare, thus making it an unsuitable marker for deep SLE remission.

Performance of timed function tests as outcome measures in idiopathic inflammatory myopathy-results from a single-centre cohort.

OBJECTIVE: To evaluate performance of timed function tests (TFTs) in assessing muscle strength and endurance as determined by Manual Muscle Testing 8 (MMT-8) and Functional Index 2 (FI-2), respectively, in idiopathic inflammatory myopathies (IIM). METHODS: This cohort study included 42 IIM patients satisfying 2017 EULAR/ACR criteria. Patients were classified as active (n = 18) or inactive disease (n = 24) based on clinical status at baseline. MMT-8, FI-2, 30 s rise from chair test, 30 s 1 kg arm rise test and 2-min walking distance (2MWD) were administered at baseline, 3 months and 6 months. Pearson rank correlation analysis and receiver operating curves were performed to assess the performance of timed function tests. RESULTS: All patients were followed up at 3 months and 39 completed 6 months' follow-up. All the three TFTs had excellent convergent (r > 0.7, P < 0.05) and divergent validity (P < 0.05). Only 2MWD had moderate to strong correlation with ΔMMT-8 at 3 and 6 months among those with active disease (P = 0.001). All the TFTs correlated with ΔFI-2 in active disease but only Δ2MWD correlated with ΔFI-2 in inactive disease at 6 months (r = 0.506, P = 0.036). At a cut-off of 5% improvement in MMT-8, 2MWD had an area under the curve (AUC) of 0.868 with 95% sensitivity with 2% improvement at 3 months. To detect a 10% ΔMMT-8, Δ2MWD at a cut of 8% and 7% had an AUC of 0.909 and 0.893 with a sensitivity of 92% at 3 and 6 months, respectively (P < 0.05). CONCLUSION: 2MWD is a reliable indicator of muscle strength, endurance and treatment response. The 2MWD can be self-administered by patients, making it a potential patient-reported outcome measure.

Atypical neurological manifestations in Wernicke's encephalopathy due to hyperemesis gravidarum.

OBJECTIVES: Hyperemesis gravidarum is known to induce nutritional, water and electrolyte deficiencies which can be fatal if not treated urgently. Thiamine deficiency may lead to a constellation of neurological symptoms that include Wernicke encephalopathy. Moreover, Wernicke encephalopathy is typically manifested as ocular paresis, ataxia and confusion. METHODS: Retrospective review of 6 women who developed neurological abnormalities following hyperemesis gravidarum and were treated with varying dosage of parenteral thiamine. RESULTS: Five women developed atypical neurological symptoms, namely, slurred speech, visual loss, seizure and aggressive behaviour while one woman developed typical clinical triad of Wernicke encephalopathy after hyperemesis gravidarum. Magnetic Resonance Imaging (MRI) scans revealed abnormalities suggestive of Wernicke encephalopathy in three women only. All women improved after parenteral thiamine administration during hospital stay and had a complete neurological recovery during 2 months follow up. DISCUSSION: Wernicke encephalopathy may not be necessarily associated with the typical neurological triad and may not have noticeable hyperintensity signal in dorsomedial thalami, mammillary bodies, hippocampus and periaqueductal region during magnetic resonance imaging. Atypical neurological signs and symptoms following hyperemesis gravidarum would invariably respond immediately to appropriate dosage of parenteral thiamine. A lower loading dosage of thiamine (100 mg thrice daily) appeared adequate for management in women with normal MRI scans.

Clinical features, severity and outcome of acute pancreatitis in systemic lupus erythematosus.

Acute pancreatitis (AP) is a rare but life threatening manifestation of Systemic Lupus Erythematosus (SLE). The current study aims to study the clinical characteristics, severity, mortality, and outcome of SLE-related AP in Indian population. We retrospectively reviewed medical records of patients with SLE who had AP in the past. Data from 13 rheumatology centers across India were compiled. All patients satisfied SLICC criteria for SLE and ATLANTA criteria for AP. AP was classified in to mild, moderate and severe using revised Atlanta classification. Patients with known risk factors like gall stone and alcohol were excluded.Sixty-six patients (six, children) were studied. Majority of patients were females (82%). The median age of presentation was 24 (11-63) years and most patients (57.5%) presented within first year of diagnosis of lupus. AP occurred mostly in the setting of active lupus (89%). Active nephritis was seen in 39% while a fourth had CNS disease. Patients with severe AP had lower C3. Ascites and sepsis were most common local and systemic complications, respectively. Mortality was 17%. Hypocalcemia, presence of sepsis and shock predicted mortality. In the multivariate analysis, only presence of shock remained as independent predictor of death (OR 63.0, 95% CI: 5.2-760.3). Pancreatitis is an early manifestation of SLE and is associated with active disease. Significant mortality is seen particularly with severe pancreatitis.

Association of Oxidative Stress with Disease Activity and Damage in Systemic Lupus Erythematosus: A Cross Sectional Study from a Tertiary Care Centre in Southern India.

To study oxidative stress in systemic lupus erythematosus (SLE) by estimating serum oxidised LDL (OxLDL), 8-hydroxy-2'-deoxyguanosine (8-OHdG), malondialdehyde (MDA), and total anti-oxidant status and to correlate with SLE disease activity and disease damage. Eighty SLE patients satisfying the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) 2012 criteria and 80 healthy controls were studied. Exclusion criteria were infections, renal insufficiency, other connective tissue diseases, drug-induced lupus, smoking, alcohol consumption. Disease activity was measured by SLE disease activity index-2 K (SLEDAI), disease damage was quantified by SLICC-Damage Index (SDI). Sera was tested for OxLDL, 8-OHdG, and total antioxidant status (TAS) by double-antibody sandwich ELISA; MDA measured by Colorimetric assay. Oxidative stress markers were compared between group1- controls, group 2-mildly active SLE (SLEDAI ≤ 5), group 3- moderate to highly active SLE (SLEDAI ≥ 6). SLE patients had significantly higher MDA, 8-OHdG and lower TAS when compared to healthy controls, while OxLDL was similar in the three groups. MDA, 8-OHdG were significantly higher, TAS lower in group 3 compared to group 2. MDA had positive correlation with SLEDAI, TAS negatively correlated with SLEDAI. SLE with neuropsychiatric manifestations, vasculitis, anti-sdDNA antibodies had higher MDA, MDA/TAS ratio. SLE patients with thrombocytopenia, and vasculitis had higher OxLDL. Only OxLDL was significantly higher in those patients who have SDI > 1. SLE patients have increased oxidative stress measured by increases in MDA, 8-OHdG, and lower total antioxidant status that was associated with disease activity and some disease manifestations. However only OxLDL was associated with damage.

Musculoskeletal problems in pregnancy.

Physiological changes during normal pregnancy are likely to give rise to musculoskeletal symptoms. On the other hand, autoimmune rheumatological diseases disproportionately affect women, often presenting during the childbearing years and sometimes during pregnancy. Contrary to autoimmune rheumatological diseases, the management of non-autoimmune musculoskeletal manifestations is generally conservative and often tends to resolve after pregnancy. It is therefore important to be aware of various musculoskeletal problems seen in the course of pregnancy. In this narrative review, commonly encountered non-autoimmune musculoskeletal problems during pregnancy have been described.

Pulmonary-renal syndromes: Experience from an Indian Intensive Care Unit.

BACKGROUND: The etiology of patients presenting with pulmonary-renal syndrome (PRS) to Intensive Care Units (ICUs) in India is not previously reported. AIMS: The aim was to describe the prevalence, etiology, clinical manifestations, and outcomes of PRS in an Indian ICU and identify variables that differentiate immunologic causes of PRS from tropical syndromes presenting with PRS. MATERIALS AND METHODS: We conducted a prospective observational study of all patients presenting with PRS over 1-year. Clinical characteristics of patients with "definite PRS" were compared with those with "PRS mimics". RESULTS: We saw 27 patients with "provisional PRS" over the said duration; this included 13 patients with "definite PRS" and 14 with "PRS mimics". The clinical symptoms were similar, but patients with PRS were younger and presented with longer symptom duration. Ninety-two percent of the PRS cohort required mechanical ventilation, 77% required vasopressors and 61.5% required dialysis within 48 h of ICU admission. The etiologic diagnosis of PRS was made after ICU admission in 61.5%. Systemic lupus erythrematosus (54%) was the most common diagnosis. A combination of biopsy and serology was needed in the majority (69%, 9/13). Pulse methylprednisolone (92%) and cyclophosphamide (61.5%) was the most common protocol employed. Patients with PRS had more alveolar hemorrhage, hypoxemia and higher mortality (69%) when compared to "PRS mimics". CONCLUSION: The spectrum of PRS is different in the tropics and tropical syndromes presenting with PRS are not uncommon. Multicentric studies are needed to further characterize the burden, etiology, treatment protocols, and outcomes of PRS in India.

Diagnosis, management and outcomes of primary hypokalemic periodic paralysis during pregnancy.

Primary hypokalaemic periodic paralysis during pregnancy has been rarely reported. Four pregnant women with the acute onset of flaccid paralysis presented between January 2018 and December 2021. Focussed history and physical examination helped an appropriate radiological and laboratory investigation plan to be made. All women recovered within 4-7 days of potassium supplementation. Supplemental potassium continued until delivery. A pain management plan with continuous epidural infusion helped in avoiding stress-induced hypokalaemia. None of the women developed an episode of muscle weakness during the intervening period. In conclusion, a focussed history and targeted laboratory investigation are needed to diagnose primary hypokalaemic periodic paralysis. Early administration of oral or intravenous potassium is crucial in improving fetomaternal outcomes.

Integrating point of care ultrasound into rheumatology practice.

A scenario-based probability approach integrating point of care rheumatology ultrasound (POCRUS) into rheumatology practice has recently been proposed as a teaching methodology to encourage greater awareness of US for practicing clinicians especially with respect to the management of overlapping clinical conditions. This article reviews the rheumatological areas where application of POCRUS substantially enhances clinical reasoning to confirm or exclude target conditions. It highlights the definitions of ultrasound-detected pathologies in rheumatoid arthritis (RA), spondylarthritis, gout and crystal arthritis, osteoarthritis, polymyalgia rheumatica and discusses the added value of POCRUS in diagnosing these conditions. It summarizes advances in predicting giant cell arteritis with sequential application of a probability score and ultrasonography. It discusses the potential for salivary gland ultrasound in the diagnostic workup of suspected Sjogren's syndrome. Other areas for POCRUS application such as in suspected nerve entrapment are reviewed.

Rheumatoid arthritis autologous synovial fluid affects the plasticity and function of peripheral and induced T regulatory cells in vitro.

The synovial fluid (SF) microenvironment in rheumatoid arthritis (RA) may alter the stability and function of Tregs. In the present study, we assessed cytokine levels and percentage of Tregs, Tregs expressing CXCR3 (Th1-like Treg), CCR6 (Th17-like Treg) in RA peripheral blood (PB) and RA-SF using fluorescence cytometry. Effect of autologous SF on plasticity and function of RA-PB Tregs (pTregs; CD4+CD25hiCD127Lo/-) and induced vimentin-pulsed Tregs (iTregsVIM) was assessed in vitro. Cytokines and percentage of Th1-like and Th17-like Tregs were higher in RA-PB than OA-PB; higher in RA-SF than osteoarthritis (OA)-SF. Compared to OA-SF exposed OA-pTregs, RA-SF exposed RA-pTregs showed higher percentage of Th1-like (11% vs 20%) and Th17-like (16% vs 36%) Tregs; higher T-bet (p = 0.0001), RORγ (p = 0.0001) and lower FOXP3 (p = 0.0001) gene expression; and diminished percentage suppression of autologous T effector cells (36% vs 74%). RA-SF exposed iTregsVIM showed increased percentage of Th1-like and Th17-like Tregs compared to iTregsVIM exposed to AB serum (8% vs 0.1%; 21% vs 0.1%). IL-2, Tocilizumab and 5-azacytidine reduced the conversion of iTregsVIM (8% vs 2.4%; 21% vs 6.9%), when used in combination. To conclude, microenvironment in the RA synovial fluid is possibly responsible for conversion of pTregs into Th-like Tregs and their functional loss. A blockade of cytokine receptors and methyl transferases could inhibit Tregs conversion, providing clinical relevance for future Tregs targeting therapies.

Conventional Tregs in treatment-naïve rheumatoid arthritis are deficient in suppressive function with an increase in percentage of CXCR3 and CCR6 expressing Tregs.

In rheumatoid arthritis (RA), immune homeostasis is maintained by T regulatory cells (Tregs) that in an inflammatory milieu can change towards T-helper-like phenotypes (Th-like Tregs). Our aim was to examine the phenotypic and functional characteristics of CD4+CD25+CD127lo/- Tregs, Th-like Tregs and T effector (Teff) cells in the peripheral blood (PB) and synovial fluid (SF) of treatment-naïve early RA, as compared to osteoarthritis (OA) and healthy control (HC) peripheral blood. Frequencies of Tregs, CXCR3, CCR6 expressing Tregs (Th-like Tregs), and Teff cells were analyzed using flow cytometry in RA (n = 80), OA (n = 20), and HC (n = 40). Cytokine concentrations of the respective T cell subsets in plasma and SF were measured using flow cytometric bead array. Tregs sorted from RA and HC PB using magnetic beads were analyzed for functional capacities by CFSE proliferation assay and FOXP3 gene expression using real-time PCR. We observed that the frequencies of Th17 cells in PB and SF were significantly higher in RA when compared to HC, whereas Tregs were lower in PB and high in SF compared to HC and OA respectively. Th1- and Th17-related pro-inflammatory cytokines IL12p70, INF-γ, TNF-α, and IL-6, and IL-17A were significantly higher in the plasma and SF of RA. Tregs expressing CXCR3 (Th1-like Tregs) and CCR6 (Th17-like Treg) were significantly higher in PB and SF of RA compared to controls and was positively associated with seropositivity and disease activity. Treg cells isolated from peripheral blood of RA showed decreased function and reduced FOXP3 gene expression compared to HC. In our study, we have demonstrated higher frequencies of Th1 and Th17 cells and increased circulatory and SF pro-inflammatory cytokines (IL12P70, INF-γ, IL-6, IL-17A, and TNF-α) in RA. This inflammatory milieu might alter total Tregs frequencies and influence conversion of Tregs into Th-like Tregs.

Low C4A copy numbers and higher HERV gene insertion contributes to increased risk of SLE, with absence of association with disease phenotype and disease activity.

Low copy numbers (CNs) of C4 genes are associated with systemic autoimmune disorders and affects autoantibody diversity and disease subgroups. The primary objective of this study was to characterize diversity of complement (C4) and C4-Human Endogenous Retrovirus (HERV) gene copy numbers in SLE. We also sought to assess the association of C4 and C4-HERV CNs with serum complement levels, autoantibodies, disease phenotypes and activity. Finally, we checked the association of C4 and HERV CNs with specific HLA alleles. Genomic DNA from 70 SLE and 90 healthy controls of south Indian Tamil origin were included. Demographic, clinical and serological data was collected in a predetermined proforma. CNs of C4A and C4B genes and the frequency of insertion of 6.4kb HERV within C4 gene (C4AL, C4BL) was determined using droplet digital polymerase chain reaction (ddPCR). A four digit high resolution HLA genotyping was done using next generation sequencing. In our cohort, the total C4 gene copies ranged from 2 to 6. Compared to controls, presence of two or less copies of C4A gene was associated with SLE risk (p = 0.005; OR = 2.79; 95% CI = 1.29-6.22). Higher frequency of HERV insertion in C4A than in C4B increases such risk (p = 0.000; OR = 12.67; 95% CI = 2.80-115.3). AL-AL-AL-BS genotype was significantly higher in controls than SLE (9%vs1%, p = 0.04; OR = 0.15, 95% CI = 0.00-0.16). Distribution of HLA alleles was not different in SLE compared to controls as well as in SLE subjects with ≤ 2 copies and > 2 copies of C4A, but HLA allele distribution was diverse in subjects with C4B ≤ 2 copies and > 2 copies. Finally, there was no correlation between the C4 and the C4-HERV diversity and complement levels, autoantibodies, disease phenotypes and activity. In conclusion, our data show that, low C4A copy number and higher insertion of HERV-K in C4A increases the risk for SLE. C4 and C4-HERV CNs did not correlate with serum complements, autoantibodies, disease phenotypes and activity in SLE. Further validation in a larger homogenous SLE cohort is needed.

Bilateral Optic Neuritis and Facial Palsy Following COVID-19 Infection.

Cases of optic neuritis have been reported following the novel coronavirus disease 2019 (COVID-19), with most being unilateral and associated with demyelinating illness. We report a case of a 22-year-old woman who presented with sudden onset painless diminution of vision in both eyes six weeks following COVID-19 infection. She also had a history of left lower motor neuron (LMN) facial palsy immediately following COVID-19 disease that recovered fully on steroids. Ocular examination and ancillary and laboratory investigations pointed to bilateral atypical optic neuritis. The patient responded well to the standard optic neuritis treatment protocol. We diagnosed her as a case of left LMN facial palsy and parainfectious bilateral optic neuritis following COVID-19. Parainfectious bilateral optic neuritis and facial nerve palsy associated with COVID-19 can occur following COVID-19 disease. Ours is the first case to report the occurrence of both in a patient.

Performance of Clinical and Biochemical Parameters in Identifying Renal Histopathology and Predictors of One-Year Renal Outcome in Lupus Nephritis-A Single Centre Study from India.

Objectives: To assess the performance of clinical and biochemical parameters in identifying renal histopathology. To assess the performance of a combination of demographic, clinical, serological and histopathological parameters in determining renal response at one year. Methods: Data of biopsy-proven (ISN/RPS­2003 criteria) Lupus Nephritis (LN) were extracted from the institute database. Demographic, clinical and biochemical parameters at the time of biopsy were noted, and their associations with histopathological class, activity and chronicity scores were evaluated. Follow-up data at one year were collected. Complete, partial or no response (CR, PR, NR) for renal outcomes at one year and the predictors of NR were assessed. Results: Out of the 333 renal biopsies, 240 (71.8%) were Class III/IV. More patients with Class III/IV LN had hypertension (52.1%) and low eGFR (p < 0.001). Among Class III/IV, AS correlated weakly with UPCR (r = 0.31, p < 0.01), eGFR (r = −0.172; p < 0.01) and CS with eGFR (r = −0.212; p < 0.01). The presence of either hypertension, UPCR > 0.5 g/day, active urinary sediments or serum creatinine >1.3 g/dL had a sensitivity of >96% and specificity of <9% in detecting proliferative LN, crescents, interstitial inflammation and chronicity. NR was higher in males (aOR:3.9, 95% CI:1.4−11.0, p < 0.001), those with abnormal baseline creatinine (aOR: 1.9, 95% CI: 1.1−3.2, p < 0.001), higher renal SLEDAI (p < 0.05), higher AS, CS (p < 0.001) and interstitial inflammation (p < 0.005). In the binary logistic regression, the combination of male sex, baseline creatinine, UPCR and CS performed best in predicting NR (AUC: 0.762; 95% CI: 0.684−0.840, p < 0.001). Conclusions: Clinical and biochemical parameters alone have a poor specificity in identifying renal histopathology. A combination of demographic, clinical and histopathology parameters can better predict renal outcomes at one year.

Effect of skin phototype on quantitative nailfold capillaroscopy.

Objectives: To determine the impact of Fitzpatrick scale-based skin phototype on visualization of capillary density using nailfold capillaroscopy in healthy Indian adults. Methods: In this cross-sectional study, healthy adults were examined for nailfold capillaroscopy findings utilizing a portable capillary microscope at 800× magnification. Photographs of two contiguous areas measuring 1 mm2 each of the distal row of capillaries were captured. Images were captured from the central area of all fingers except thumb in both hands. Capillary density and morphology of nailfold capillaroscopies were assessed by two blinded assessors. The nailfold capillaroscopy parameters were compared between the Standard Fitzpatrick scale-based skin phototypes. Results: A total of 118 healthy adults were enrolled in the study. Type III, IV, V, and VI skin phototypes were seen in 27 (22.90%), 32 (27.19%), 29 (24.58%), and 30 (25.42%) participants, respectively. All participants (100%) had normal nailfold capillaroscopy morphology and architecture. Zero capillaries were visible in 11 fingers among 5 patients (4.24%) and all of them had Type VI phototype. The median capillary density per mm was 5.19 (interquartile range = 4.37-6.75) with 90 (76.27%) participants having less than seven capillaries. The median average capillary density was significantly different (p-value < 0.0001) across Type III (8.13, interquartile range = 6.44-8.88), Type IV (5.67, interquartile range = 4.41-6.98), Type V (4.94, interquartile range = 4.19-5.38), and Type VI (4.53, interquartile range = 3.72-4.91) phototypes (p < 0.05). Conclusion: The number of capillaries visualized during nailfold capillaroscopy decreases as the skin pigmentation increases. There is a need to redefine the nailfold capillaroscopy density and avascularity by taking skin phototype as one of the determinants before labeling a nailfold capillaroscopy finding with less visualized capillaries as abnormal.

Corneal ulcer in reactive arthritis.

A 20-year-old male presented with acute lower limb oligoarthritis and enthesitis followed by acute onset redness, watering, pain and decreased vision in the right eye. He had recent history of diarrhoea with fever. Erythrocyte sedimentation rate and high-sensitivity C-reactive protein (hsCRP) were raised and human leukocyte antigen-B27 was positive. The best corrected visual acuity (BCVA) in the right eye was 20/120 and it showed a paracentral shallow corneal ulcer of size 3 × 4 mm with underlying dense stromal infiltrates and haze. Microbiological evaluation of corneal scrapings was reported as Staphylococcus hominis. The epithelium healed on topical antibiotics in one week, but there were persistent punctate erosions and pleomorphic anterior stromal infiltrates and haze. The residual keratitis healed completely on topical steroids in ten days, with BCVA improving to 20/20. A diagnosis of reactive arthritis with immune-mediated keratitis was made.