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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]

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]

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]

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]

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 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]

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.