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Objectives: Currently, there is limited data regarding the outcomes of lupus nephritis (LN) with moderate to severe renal failure at presentation (defined by low glomerular filtration rate; GFR <30 mL/min). Methods: Sixty‐four patients with biopsy‐proven LN and estimated GFR (eGFR) <30 mL/min were prospectively analyzed. Outcome measure of persistently low eGFR, end‐stage renal disease (ESRD) or death at 365 days were grouped as Major Adverse Kidney Events (MAKE365). Results: Diagnosis of lupus was simultaneous with onset of renal disease in 60% of cases. Histologically, 82.3% (n = 51) were class IV, the median serum creatinine was 4 mg/dL (interquartile range [IQR] 3.1‐5.9 mg/dL), median eGFR was 13.75 mL/min (IQR 9.25‐19 mL/min) and 42.2% (n = 27) required dialysis at presentation. Induction regimens included National Institute of Health (68.2%), Eurolupus protocol (10.9%) and mycophenolate mofetil (8%). Over 365 days, 23 (37.5%) subjects died, while 41 (62.5%) survived. The majority of deaths were due to infection and sepsis (14/23). Among the survivors, 70.7% had good renal outcome, 12.1% had persistently low GFR (<30 mL/min), while 17% developed ESRD. In this group, treatment response rate was 84.6% (complete response 25.6%, partial response 59%). Those with a better renal function at presentation had a good treatment response (100% vs. 40%). Altogether, n = 35 (54.6%) were included in the MAKE365 category. Between the renal survival group (n = 29) versus the MAKE365 group (n = 35) there was no difference in clinical or histological parameters. Conclusion: The current treatment protocols had a good response rate in patients with LN even with severe kidney injury at presentation. However, the risk of serious infections and subsequent mortality was high. [ABSTRACT FROM AUTHOR]

Objectives: Currently, there is limited data regarding the outcomes of lupus nephritis (LN) with moderate to severe renal failure at presentation (defined by low glomerular filtration rate; GFR <30 mL/min). Methods: Sixty‐four patients with biopsy‐proven LN and estimated GFR (eGFR) <30 mL/min were prospectively analyzed. Outcome measure of persistently low eGFR, end‐stage renal disease (ESRD) or death at 365 days were grouped as Major Adverse Kidney Events (MAKE365). Results: Diagnosis of lupus was simultaneous with onset of renal disease in 60% of cases. Histologically, 82.3% (n = 51) were class IV, the median serum creatinine was 4 mg/dL (interquartile range [IQR] 3.1‐5.9 mg/dL), median eGFR was 13.75 mL/min (IQR 9.25‐19 mL/min) and 42.2% (n = 27) required dialysis at presentation. Induction regimens included National Institute of Health (68.2%), Eurolupus protocol (10.9%) and mycophenolate mofetil (8%). Over 365 days, 23 (37.5%) subjects died, while 41 (62.5%) survived. The majority of deaths were due to infection and sepsis (14/23). Among the survivors, 70.7% had good renal outcome, 12.1% had persistently low GFR (<30 mL/min), while 17% developed ESRD. In this group, treatment response rate was 84.6% (complete response 25.6%, partial response 59%). Those with a better renal function at presentation had a good treatment response (100% vs. 40%). Altogether, n = 35 (54.6%) were included in the MAKE365 category. Between the renal survival group (n = 29) versus the MAKE365 group (n = 35) there was no difference in clinical or histological parameters. Conclusion: The current treatment protocols had a good response rate in patients with LN even with severe kidney injury at presentation. However, the risk of serious infections and subsequent mortality was high. [ABSTRACT FROM AUTHOR]

Renal involvement is a common occurrence in subjects with rheumatological diseases and can develop either due to the disease itself or secondary to drugs used in the treatment. The prevalence of renal involvement and its severity depends on the underlying disease as well as aggressiveness of the therapy. For most rheumatological diseases, renal involvement heralds a poor prognosis and warrants aggressive immunosuppressive treatment. Thus, it is important to diagnose and manage them at an early stage. On the other hand, patients with primary kidney disease can also develop rheumatological manifestations which need to be differentiated from the former. This article provides the nephrologist's perspective upon various rheumatological disorders and associated renal involvement with the aim of sensitizing the rheumatological community about them, resulting in better management of these subjects. [ABSTRACT FROM AUTHOR]

Extracorporeal treatments have an important role in the management of several types of poisonings and drug overdosage. Methanol poisoning is a major problem in large parts of the developing world, especially among the economically depressed, and is responsible for innumerable deaths annually. Poisoning occurs when methanol, a contaminant of bootlegged alcohol, is consumed unknowingly. Toxicity is related to formaldehyde and formic acid formed as a result of methanol metabolism, and presents as metabolic acidosis, visual impairment, neurological manifestations, and shock. Initial symptoms are nonspecific and masked by the inebriating effect of ethanol. Appropriate management requires immediate administration of ethanol, which competitively inhibits methanol metabolism and prevents the generation of toxic formic acid and hemodialysis to achieve expeditious clearance of methanol and formic acid. Fomepizole, a safer metabolic inhibitor, has largely replaced ethanol in the western world. Delay in seeking medical attention contributes to mortality and morbidity. We report on a patient who presented to us 8 hr after consumption of countrymade alcohol with symptoms of methanol poisoning. Prompt administration of ethanol and institution of hemodialysis resulted in complete reversal of all manifestations. [ABSTRACT FROM AUTHOR]

The relationship between C-reactive protein (CRP), a marker of subclinical inflammation, and intramyocellular lipid (IMCL) content, a novel correlate of insulin resistance, has not previously been investigated. We estimated IMCL content in soleus muscle in 30 healthy Asian Indian males using proton magnetic resonance spectroscopy (1H MRS), and correlated it with body mass index (BMI), measures of abdominal obesity, percentage of body fat (%BF), serum lipoproteins, fasting and post-oral glucose load serum insulin levels and other surrogate markers of insulin resistance. Soleus muscle IMCL content was significantly correlated with age (ρ = 0·64, P < 0·001), BMI (ρ = 0·41, P < 0·05), %BF (ρ = 0·53, P ≤ 0·01), waist circumference (ρ = 0·45, P < 0·05) and waist-to-hip circumference ratio (ρ = 0·58, P < 0·01) but did not correlate significantly with insulin resistance measured by the homeostasis model assessment (HOMA-IR) or CRP levels. CRP levels did not correlate with the HOMA-IR value. Soleus muscle IMCL content correlated significantly with measures of generalized and abdominal obesity but not with insulin sensitivity or CRP levels in healthy Asian Indian males. Studies are needed in other ethnic groups to corroborate these data. [ABSTRACT FROM AUTHOR]

Aim: Antineutrophil cytoplasmic antibody‐associated vasculitides (AAV) are a group of small vessel vasculitis with systemic presentations and considerable morbidity and mortality. Pregnancy in these patients poses a significant therapeutic challenge. There is limited published literature regarding pregnancy in AAV. Methods: Two cases of successful pregnancy outcomes in patients with active AAV are described. A systematic review was conducted on the lines of the PRISMA statement for conducting systemic reviews: PubMed (inception of PubMed until 30 April 2017, English language only) and EmBase databases were searched using the following terms: 'pregnancy' AND 'ANCA associated vasculitis' OR 'granulomatosis with polyangiitis' OR 'eosinophilic granulomatosis with polyangiitis' OR 'microscopic polyangiitis' OR 'Churg‐Strauss syndrome' OR 'Wegener's granulomatosis'. Results: One hundred and thirty‐seven pregnancies were documented in 110 patients of AAV. Vasculitis diagnosis was made before pregnancy in 69, during pregnancy in 32 and after pregnancy in 9 patients. Mean age at the time of pregnancy was 29.3 ± 5.3 years. There were 91 term pregnancies, 28 were preterm pregnancies, 15 abortions and 3 still births; 78 had normal delivery and 26 had caesarian section. Conclusion: Successful pregnancies have been reported in AAV patients. [ABSTRACT FROM AUTHOR]

Abstract: 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. [ABSTRACT FROM AUTHOR]

Aim: To study the expression of B cell‐activating factor of tumor necrosis factor family (BAFF) and A proliferation‐inducing ligand (APRIL) genes in active and remitting patients with anti‐neutrophil cytoplasmic antibody‐associated vasculitis (AAV) and healthy controls and their correlation with disease activity. Methods: This was a prospective case‐control study. Gene expressions of BAFF and APRIL were studied in 32 patients with AAV (16 each with active disease and in remission) and 30 healthy age and sex matched controls by real‐time polymerase chain reaction. Results: Out of 32 AAV patients, 26 had granulomatosis with polyangiitis (GPA) and 6 had microscopic polyangiitis (MPA). Mean ages of patients in active (12 GPA and 4 MPA) and remission (14 GPA and 2 MPA) groups were 39.4 ± 17.2 and 44.6 ± 16.1 years, respectively. BAFF gene expression was significantly higher in both the active AAV group and remission AAV group compared to controls (P <.01). The BAFF expression was significantly higher in AAV patients in remission compared to active AAV patients (P =.003). In contrast, APRIL expression did not differ between AAV patients and controls (P =.829). However, APRIL had significantly higher expression in remission as compared to active patients (P =.048). There was no significant correlation of both BAFF and APRIL expression with disease activity markers (erythrocyte sedimentation rate, C‐reactive protein, platelets and Birmingham Vasculitis Activity Score version 3). Conclusion: BAFF gene is significantly expressed in patients with AAV. Among AAV patients, there is a significantly higher expression of BAFF and APRIL in remitting state of the disease as compared to active state. There is no significant change in APRIL gene expression in patients with AAV as compared to controls. This makes a case for anti‐BAFF therapy in future for AAV patients in northern India. [ABSTRACT FROM AUTHOR]

Introduction: Rifampicin is one of the most effective components of anti‐tuberculous therapy (ATT). Since rifampicin is a hepatic enzyme (CYP3A4) inducer, in a post‐renal transplant recipient, the dose of calcineurin inhibitors needs to be up‐regulated and frequently monitored. In resource‐limited (low‐ and lower‐middle‐income countries) setting this is not always feasible. Therefore, we evaluated a non‐rifampicin‐based ATT using levofloxacin in kidney transplant recipients. Methods: We retrospectively studied the medical records of renal transplant recipients diagnosed with tuberculosis in our institute between 2014 and 2017. After a brief discussion with patients regarding the nature and course of ATT, those who opted for a non‐rifampicin based therapy due to financial constraints were included in the study and followed for a minimum of 6 months period after the completion of ATT. Results: Out of the 550 renal transplant recipients, 67 (12.2%) developed tuberculosis after a median period of 24 (1‐228) months following transplantation, of them, 64 patients opted for non‐rifampicin‐based ATT. The mean age was 37.6 years. Only 25% were given anti‐thymocyte globulin based induction, while the majority (56; 87.5%) of them were on tacrolimus‐based triple‐drug maintenance therapy. Extrapulmonary tuberculosis was noted in 33% of cases, while 12 (18.7%) had disseminated disease. The median duration of treatment was 12 months and the cure rate of 93.7% (n = 60) was achieved at the end of therapy. Conclusion: Levofloxacin based ATT appears to be a safe and effective alternative of rifampicin in kidney transplant recipients who cannot afford heightened tacrolimus dosage. SUMMARY AT A GLANCE: In this single‐centre study, the authors report their experience using levofloxacin instead of rifampicin for the treatment of tuberculosis after kidney transplantation. Therapy with levofloxacin appeared safe and effective, and may provide a more cost‐effective treatment for post‐transplant tuberculosis in financially constrained clinical settings. [ABSTRACT FROM AUTHOR]

Background: Collapsing focal segmental glomerulosclerosis (cFSGS) is an aggressive glomerular disease presenting as a nephrotic syndrome that has lower rates of remission with conventional immunosuppressive therapy and rapidly progresses to end‐stage‐renal‐disease (ESRD). We report eight cases of HIV‐negative cFSGS treated with rituximab. Methods: The current report is a retrospective case series of cFSGS treated with rituximab from January 2011 to March 2020, at varying phases of the disease. Results: Eight out of the 70 cFSGS patients received rituximab. The median age of patients was 30 years (IQR 24.25‐37.5); five patients were males. The median serum creatinine, mean serum albumin and median 24 hours urinary protein at presentation was 0.9 (IQR 0.66‐1.27) mg/dL, 2.95 ± 1.15 g/dL, 4.87 (IQR 1.64‐5.75) g/day, respectively. Two patients were steroid‐resistant, one steroid and tacrolimus dependent, one steroid and cyclosporine dependent, two steroids and tacrolimus resistant, one steroid, tacrolimus, cyclophosphamide, mycophenolate mofetil resistant and one steroid‐resistant and tacrolimus dependent before rituximab therapy. Rituximab was given either as targeted therapy (after an initial dose of 375 mg/m2; patients having CD‐19 levels >5/μL or >1% at 1 month received additional low‐dose [100 mg] of rituximab), or weekly regimen. Five patients received CD‐19 targeted rituximab; three received weekly doses of 375 mg/m2, cumulative doses being 820 ± 228.03 mg, and 1800 ± 721.11 mg, respectively. At the end of median follow‐up of 15 months, five (62.5%) patients were in remission (three partial, two complete remissions), two (25%) were resistant to therapy; one (12.5%) progressed to ESRD. Conclusion: Rituximab is reasonably safe and achieves/maintains remission in 60% of cFSGS cases. SUMMARY AT A GLANCE: This is a retrospective study reporting the effects of Rituximab on 8 patients with the collapsing form of focal segmental glomerulosclerosis. Whilst retrospective in nature, the study suggested that rituximab might be a promising form of treatment for this condition, which is traditionally resistant to immunosuppression therapy, resulting in a remission rate of 60% with a reasonable safety profile. [ABSTRACT FROM AUTHOR]

Alport syndrome (AS) is an inherited disorder of basement membranes caused by mutations affecting specific proteins of the type IV collagen family, presenting with nephropathy and extrarenal manifestations such as sensorineural deafness and ocular anomalies. Ten percentage to 15% of the patients with AS have autosomal recessive (ARAS) due to mutation in either COL4A3 or COL4A4 gene. We report a novel mutation in the COL4A3 gene in an Indian family with ARAS. The above‐mentioned genetic anomaly was a missense variation in exon 26 of the COL4A3 gene (chr2:228137797G>A; c.1891G>A) that resulted in the amino acid substitution of Arginine for Glycine at codon 631 (p.Gly631Arg) that was present in the heterozygous state in the asymptomatic parents and homozygous state in the male offspring who presented with early‐onset end‐stage renal disease, lenticonus and hearing loss. The patient (male offspring) underwent successful renal transplantation with his mother as a donor. SUMMARY AT A GLANCE: A novel mutation in the COL4A3 gene in an Indian family with autosomal recessive Alport syndrome is described. The index case underwent a successful renal transplantation with his mother (who possessed the associated missense mutation) as the donor. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: End points currently used in lupus nephritis (LN) clinical trials lack uniformity and questionably reflect long‐term kidney survival. This study was undertaken to identify short‐term end points that predict long‐term kidney outcomes for use in clinical trials. Methods: A database of 944 patients with LN was assembled from 3 clinical trials and 12 longitudinal cohorts. Variables from the first 12 months of treatment after diagnosis of active LN (prediction period) were assessed as potential predictors of long‐term outcomes in a 36‐month follow‐up period. The long‐term outcomes examined were new or progressive chronic kidney disease (CKD), severe kidney injury (SKI), and the need for permanent renal replacement therapy (RRT). To predict the risk for each outcome, hazard index tools (HITs) were derived using multivariable analysis with Cox proportional hazards regression. Results: Among 550 eligible subjects, 54 CKD, 55 SKI, and 22 RRT events occurred. Variables in the final CKD HIT were prediction‐period CKD status, 12‐month proteinuria, and 12‐month serum creatinine level. The SKI HIT variables included prediction‐period CKD status, International Society of Nephrology (ISN)/Renal Pathology Society (RPS) class, 12‐month proteinuria, 12‐month serum creatinine level, race, and an interaction between ISN/RPS class and 12‐month proteinuria. The RRT HIT included age at diagnosis, 12‐month proteinuria, and 12‐month serum creatinine level. Each HIT validated well internally (c‐indices 0.84–0.92) and in an independent LN cohort (c‐indices 0.89–0.92). Conclusion: HITs, derived from short‐term kidney responses to treatment, correlate with long‐term kidney outcomes, and now must be validated as surrogate end points for LN clinical trials. [ABSTRACT FROM AUTHOR]

The incidence and clinical features of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) have been shown to vary according to geographical areas, with granulomatosis with polyangiitis (GPA) being more common in northern Europe and microscopic polyangiitis (MPA) being more common in Asian countries. The annual incidence of GPA among Asians varies between 0.37/million to 2.1/million population. The prevalence of GPA has been estimated to be 1.94/100 000 in a Chinese population. Polymorphisms in class II major histocompatibility genes and ETS1 proto‐oncogene has been shown in Asian patients with GPA. There is a difference in mean age at onset and proteinase 3 (PR3) or myeloperoxidase (MPO) positivity in GPA patients from different Asian countries. Those from India had mean age of 40 years and those from Japan had mean age of 65 years. Sixty percent of GPA patients from China and Japan were MPO ANCA positive while the majority of patients from India and Korea were PR3 positive. Geographical variation with lower frequency of renal involvement in Indian studies and higher frequency in Chinese patients has also been noted. Treatment outcomes have been similar to those reported from other parts of the world. Remission was achieved in about two‐thirds of patients while relapses were noted in one‐third to half of the patients. Apart from minor differences in the organ systems involved, MPO‐ANCA GPA and PR3‐ANCA GPA had similar rates of remission and relapses. [ABSTRACT FROM AUTHOR]

Abstract: The literature on membranous nephropathy (MN) with monoclonal deposits on immunofluorescence (IF) and their outcome is very scarce. We report our experience of managing five patients with this clinical entity. The mean age of the patients was 33.2 ± 6.55 years. The mean proteinuria, serum albumin and serum creatinine was 5.73 ± 2.17 g/day, 2.86 ± 0.51 g/dL and 1.34 ± 1.19 mg/dL, respectively. None of the patients had a lymphoproliferative disorder. Only one patient had an elevated free light chain ratio. Four (80%) patients were M‐type phospholipase A2 receptor (PLA2R) negative (tissue and serum), and one (20%) was PLA2R related. Three (60%) cases had monoclonal IgG3/k, one IgG3/λ, whereas one patient with PLA2R positivity had an IgG3/IgG4k subtype. Two (67%) patients treated with cyclical cyclophosphamide and steroids (cCYC/GC) achieved complete remission and one patient (33%) with elevated baseline creatinine had a reduction in serum creatinine with persistent proteinuria at the end of the 12th month of follow‐up. One patient with PLA2R positive MN was treated with Rituximab and is in complete remission. The patient with an elevated free light chain at baseline was treated with Bortezomib/Thalidomide/Dexamethasone, had complete remission at 12 months, however, had a progressive rise in creatinine over the next 40 months of follow‐up. The current series, though limited by numbers, documents the efficacy of conventional therapies in non‐malignant associated MN with monoclonal deposits on IF. [ABSTRACT FROM AUTHOR]

Aim Primary membranous nephropathy (PMN) accounts for only 1-2% of nephrotic syndrome in children. Antibodies to m-type phospholipase A2 receptor (aPLA2R) is seen in 70% of adult PMN cases. The present study was undertaken to study m-type phospholipase A2 receptor (PLA2R) status and clinical behavior in adolescent PMN cases. Methods The present prospective observational study included adolescent (10-19 years) onset biopsy proved PMN. Patients were followed on a monthly basis with urine protein, serum albumin and creatinine. Serum aPLA2R was done at baseline and at 6 and 12 months of starting treatment. Patients were treated as per unit's protocol. Results During the study period a total of 18 patients were enrolled. The mean age of the cases was 16.27 ± 2.39 (11-19) years. Seventeen (94.44%) patients presented with nephrotic syndrome. The mean proteinuria and serum albumin was 4.52 ± 1.93 (2.43-9.20) g/day and 2.1 ± 0.6 (1.1-3.4) g/dL respectively. PMN was PLA2R related in 83%. aPLA2R and enhanced staining for PLA2R in glomeruli was seen in 14 (77.78%) and 13 (72.22%), respectively. Clinical remission at the end of 6 and 12 months of therapy was seen in 11 (61.11%) and 9 (50%) subjects respectively. There was a significant association of aPLA2R to clinical remission/ resistance. Conclusion Primary membranous nephropathy in adolescent population is aPLA2R related in over three-quarters of the cases and the response to therapy is seen in only half of them. aPLA2R monitoring is clinically relevant and should be incorporated in the management of adolescent onset PMN. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aims Immunoglobulin-G4 (IgG4)-related tubulo-interstitial nephritis (IgG4 TIN) could be the first presentation of IgG4-related systemic disease. Most of the data is from the West or Japan and retrospective, with good patient outcome. Methods This study was carried out from April 2011 to July 2013. We report a prospective follow-up of 11 patients who presented with renal dysfunction and had histological diagnosis of IgG4 TIN followed for a minimum period of 1 year or until end-stage renal disease. Results IgG4 TIN constituted 0.28% of total renal biopsies and 6.5% of all tubulointerstitial nephritis. Patient ages ranged between 21 and 71 years with a male predominance. All the patients had renal dysfunction at presentation with a mean serum creatinine of 5.12 mg/dL. Proteinuria was subnephrotic except when there was coexisting membranous glomerulonephritis (36.4%). The mean 24-h urine protien excretion was 1.8 g. Serum IgG4 levels were elevated in 10 (90.9%) patients. Ten (90.9%) patients had renomegaly and one (9.1%) had focal renal mass. Extra-renal manifestations were present in seven (63.6%). Renal histology showed pattern A in five (45.5%), pattern B in four (36.3%) and pattern C in two (18.1%) patients. All but one patient (90.9%) received immunosuppressive therapy. Four (36.3%) achieved complete remission and three (27.2%) progressed to end stage renal disease. Two patients died due to infections while on steroid therapy. One patient with a mass had end stage renal disease for 12 months and did not improve with steroid therapy, and one (pattern C) had progressive chronic kidney disease on follow-up. Conclusion IgG4 TIN in an Indian cohort most often presents with rapidly progressive renal failure and less often has extra-renal organ involvement. On follow-up, patients can experience a more aggressive course with progression to end stage renal disease. [ABSTRACT FROM AUTHOR]

Aim There have been very few studies comparing cyclophosphamide ( CTX) and calcineurin inhibitor based regimens in the management of non-immunosuppressive symptomatic therapy ( NIST) resistant idiopathic membranous nephropathy ( IMN). The present study was aimed at comparing the efficacy and safety of tacrolimus ( TAC)/steroids with cyclical CTX/steroids ( Modified Ponticelli regimen ( MPR)) in patients with IMN. Methods Idiopathic membranous nephropathy patients ( n = 70) with persistent nephrotic syndrome after at least 6 months of antiproteinuric therapy or with complications of nephrotic syndrome were equally randomized to receive TAC with oral prednisolone ( TAC*) or MPR. Antibodies against m-type phospholipase A2 receptor ( PLA2R Ab) were tested for at baseline and, at 6 and 12 months after the start of therapy. The primary end point was achievement of remission and secondary objectives were adverse effects and estimated glomerular filtration rate in both the study groups. Results Intention-to-treat analysis showed that remissions at the end of 6 (74% with TAC* vs. 60% with MPR; P = 0.30) and 12 months (71% with TAC* vs. 77% with MPR; P = 0.78) were comparable. PLA2R Ab titres at 6/12 months correlated with urine protein (r 0.54/0.58) and serum albumin (r −0.49/−0.53) at the end of therapy. Patients on CTX had a significantly higher risk of amenorrhea and while those on TAC had a greater risk of reversible nephrotoxicity. Conclusion In NIST refractory IMN, both TAC* and MPR are comparable, but with different adverse effect profile. PLA2R Ab has a very good association with proteinuria, and should be regularly monitored on clinical follow-up. [ABSTRACT FROM AUTHOR]

Aim A significant proportion of pauci-immune glomerulonephritis ( PIGN) patients are reported to have absence of anti-neutrophilic cytoplasmic antibodies ( ANCA). However, studies are controversial regarding their significance and there is limited data after the new prognostic classification of PIGN. Methods Renal biopsy-proven cases of PIGN were included and their clinical details, ANCA status by immunofluorescence ( IIF) and enzyme-linked immunosorbent assay ( ELISA), Birmingham Vasculitis Activity Score ( BVAS) and treatment outcomes at 6 months were noted. The renal biopsies were classified according to the proposed histopathological classification. Scoring was done from 0-3 for interstitial edema, interstitial fibrosis and tubular atrophy ( IFTA), interstitial inflammation and arteriosclerosis. The percentage of glomeruli with sclerosis, cellular and fibrous crescents, and percentage of subjects with glomerulitis, tuft necrosis, interstitial granuloma and vasculitis were noted. Results Out of the 84 subjects included in the study, 33 (39.3%) were negative for ANCA by both IIF and ELISA. These subjects had significantly higher renal involvement, less extra-renal manifestations and lower BVAS. On histology, they had significantly higher proportion of crescentic class (66.7% vs. 41.2%, P = 0.039), higher number of cellular crescents (66.12% vs. 53.3%, P = 0.00008), higher IFTA (1.53 vs. 1.02, P = 0.009) and less interstitial edema (1.44 vs. 1.96, P = 0.003). The treatment outcomes were worse in ANCA-negative PIGN subjects, with significantly less improvement (37.2% vs. 62.8%, P = 0.02), more deterioration (40.7% vs. 14%, P = 0.006), and reduced probability of becoming dialysis free (31.6% vs. 69.6% P = 0.009). Conclusions A negative ANCA in PIGN is associated with crescentic class, more IFTA and poor treatment outcomes. [ABSTRACT FROM AUTHOR]

Aim Many patients with systemic necrotizing vasculitis ( SNV) satisfy classification criteria of different disease entities when different classification systems are used. A new classification algorithm has been proposed recently by using the American College of Rheumatology criteria, Chapel Hill Consensus Criteria ( CHCC) and Sorensen surrogate markers for a more uniform classification of patients suffering from these rare disorders. Methods We applied this algorithm to patients diagnosed as having systemic vasculitis between 2007 and 2011. We also analyzed the data using this algorithm by incorporating the recently proposed revised CHCC nomenclature of vasculitis in place of the older criteria. Results Seventy-nine patients with SNV were studied. One patient diagnosed as microscopic polyangiitis ( MPA) had to be excluded from analysis as she had previously been diagnosed as having Behcet's disease. All patients of eosinophilic granulomatosis with polyangiitis ( EGPA), granulomatosis with polyangiitis ( GPA) and MPA were reclassified to the same diagnostic subcategory after application of the algorithm. Three (16.7%) of 18 polyarteritis nodosa patients were unclassifiable after application of the consensus algorithm while two (11.1%) were reclassified as MPA. All previously unclassifiable patients could be classified either as MPA or GPA after application of the new algorithm. There was no difference in the results when the CHCC 2012 nomenclature was used instead of the older CHCC in the consensus algorithm. Conclusion The new classification algorithm is a reliable method for classification of SNV for epidemiological purposes in our population. [ABSTRACT FROM AUTHOR]

Whether or not completing the hepatitis B vaccination in patients who have undergone kidney transplantation in the middle of incomplete vaccination schedule leads to development of protective antibody titres is not known. This study was designed to determine whether the strategy of completing hepatitis B virus (HBV) vaccination after transplantation is efficacious. Sixty-four end-stage renal disease (ESRD) patients were screened for hepatitis B surface antigen (HBsAg), antibodies to hepatitis B surface antigen (anti-HBs), hepatitis B e-antigen (HBeAg) and HBV DNA. HBsAg negative patients received four doses of 40 µg recombinant HBV vaccine. Schedule was continued in after transplantation period if it was incomplete before transplant. Anti-Hbs titres were evaluated at 1, 3, 6, 9 and 12 months. Past HBV infection was noted in 12 patients: 10 by serology plus viraemia and two by viraemia alone. Of the 46 patients without current or past HBV infection who had received at least two doses of the vaccine before transplant, 17 each had received two and three doses and 12 had completed the schedule. Seventeen (37%) exhibited protective titres. Patients who had completed vaccination were more likely to have protective titres than those incompletely vaccinated ( P = 0.02). Five patients responded to post-transplant vaccination. Partially vaccinated patients do not mount an adequate antibody response despite continued vaccination in the post-transplant period, whereas complete vaccination provides protection in 60%. The present study data highlights the need of administration of a full schedule of HBV vaccination before kidney transplantation. Nucleic acid-based tests can identify occult HBV infection. Patients partially vaccinated against HBV do not mount an adequate antibody response despite continued vaccination in the post-transplant period. The data presented here highlights the need for administration of a full schedule of vaccination before kidney transplantation for maximal protection against HBV infection. [ABSTRACT FROM AUTHOR]

Impaired cell‐mediated, as well as antibody‐mediated immunity predisposes a renal transplant recipient to a wide variety of atypical infection. With an increasing number of re‐transplant, the balance between immunosuppression and the risk of recurrent disease poses a clinical and therapeutic challenge. Here, we report a successful re‐transplantation in a case of parvovirus B19 infection leading to anaemia and collapsing glomerulopathy in the allograft managed with intravenous immunoglobulin (IVIG) and reduction of immunosuppression. This case emphasizes re‐consideration to renal transplant after clearance of the virus in a previous renal allograft lost to PVB19 infection. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 63-year old man with granulomatosis with polyangiitis (GPA) who had been diagnosed with GPA 3.5 years ago when he suffered from sinusitis, nasal crusting, microscopic hematuria and renal dysfunction. He was put on prednisolone and azathioprine. The article discusses Pyomyositis, a purulent disease and its various risk factors.