Efficacy and safety of low-dose corticosteroid with azathioprine in the treatment of elderly-onset IgA vasculitis with nephritis

2021 ◽  
Vol 14 (6) ◽  
pp. e241422
Author(s):  
Hikaru Sugimoto ◽  
Yasuko Yamaguchi ◽  
Wako Yumura ◽  
Takashi Takei
Keyword(s):  
Renal Failure ◽  
Complete Remission ◽  
Low Dose ◽  
Renal Involvement ◽  
Iga Vasculitis ◽  
Efficacy And Safety ◽  
Elderly Onset ◽  
Dose Corticosteroid ◽  
Frequent Presence

The diagnosis of elderly-onset IgA vasculitis (IgAV) and its prognosis can be difficult to ascertain because of its rarity and the frequent presence of comorbidities. Furthermore, the treatment of elderly-onset IgAV remains controversial. We report a case of IgAV in an 87-year-old patient. Renal involvement was detected early during the IgAV follow-up. He was treated with low-dose corticosteroid and azathioprine, which led to a complete remission without any adverse effects. This suggests that precise intervention with early diagnosis and careful renal follow-up may prevent renal failure and that low-dose steroids with azathioprine can be an effective treatment for elderly-onset IgAV with nephritis.

scholarly journals Low-dose corticosteroid with mizoribine might be an effective therapy for elderly-onset ISKDC grade VI IgA vasculitis

2020 ◽  
Author(s):  
Hikaru Sugimoto ◽  
Shiho Matsuno ◽  
Noriko Yamanaka ◽  
Wako Yumura ◽  
Mitsuyo Itabashi ◽  
...  
Keyword(s):  
Low Dose ◽  
Effective Therapy ◽  
Iga Vasculitis ◽  
Elderly Onset ◽  
Dose Corticosteroid

scholarly journals Complete Remission of Relapsed Hodgkin’s Lymphoma following Brentuximab Vedotin and Gemcitabine Combination Therapy with Severe Hypotension as Possible Treatment-Related Adverse Event: A Case Report

2020 ◽  
Vol 13 (1) ◽  
pp. 341-346 ◽  
Author(s):  
Ibnu Purwanto ◽  
Bambang P. Utomo ◽  
Ahmad Ghozali
Keyword(s):  
Adverse Event ◽  
Complete Remission ◽  
Hodgkin’S Lymphoma ◽  
Brentuximab Vedotin ◽  
Hodgkin's Lymphoma ◽  
High Dose ◽  
High Dose Corticosteroid ◽  
Severe Hypotension ◽  
Dose Corticosteroid

A 40-year-old Asian female with heavily treated relapsed Hodgkin’s lymphoma showed complete remission (CR) after receiving 8 cycles of brentuximab vedotin (BV) in combination with gemcitabine as 4th line treatment. The patient remained in CR at the 18-month post-treatment follow-up. She developed severe hypotension (50/36 mm Hg) with upper and lower limb petechiae and edema after the addition of gemcitabine on the 6th cycle of BV. This adverse event resolved after 3 days of treatment with vasopressor and high-dose corticosteroid. The addition of dexamethasone for the subsequent 2 cycles successfully prevented this adverse event from recurring.

Efficacy of Low Dose Clofarabine Plus Intermediate Dose Cytarabine In An Unselected AML Refractory/Relapsed Adult Population

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4349-4349
Author(s):  
Claudio Romani ◽  
Emanuele Angelucci ◽  
Barbara Scappini ◽  
Mario Petrini ◽  
Martina Pettinau ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Complete Remission ◽  
Nucleoside Analogue ◽  
Myeloid Leukemia ◽  
Low Dose ◽  
Haematological Toxicity ◽  
Allogeneic Transplantation ◽  
Gemtuzumab Ozogamicin ◽  
Acute Myeloid

Abstract Abstract 4349 Advanced AML in adult patients is a difficult challenge for hematologists with several unsuccessful approaches attempted during the last years. We tested the combination of the new nucleoside analogue Clofarabine in association with Cytarabine in a consecutive series of adult patients with advanced acute myeloid leukemia. From April 2007 to April 2010, 29 unselected advanced disease (not M3) acute myeloid leukemia (AML) patients were included in this multicenter study. There were 14 female and 15 male. Median age was 44 years (range 22–65). Twenty patients were in second or more advanced relapse and 9 had refractory disease to one or more chemotherapy regimens. Patients were treated with one cycle of five days clofarabine 22,5 mg/m2/day together with five days cytarabine 1 g/m2/day. The low dose (22,5 mg/m2) clofarabine was selected because of the burden of therapy that our patients had already received. In 19 patients gemtuzumab ozogamicin 6 mg/m2 was added by Centre physician decision. Toxicity and remission status were defined according with WHO criteria. Partial remission was considered as improvement of peripheral blood count and reduction of bone marrow blasts > 50%). Toxicity: Grade IV haematology toxicity was recorded in all patients. Extra haematological toxicity was as follows: four patients presented ≥ grade III cutaneous toxicity, ten hepatic toxicity; three patients mucositis. Eight patients experienced a life threatening infection while in aplasia (six systemic septicemia and two pneumonia) which were ultimately fatal in four. Disease response: overall, 16 over 29 patients (55%) entered in complete remission (CR) and 4 in partial remission. Overall response rate was 69%. Outcome: over the sixteen patients who entered complete remission: 1 was lost at follow up, 1 relapsed, 1 was treated with azacitidine and 2 with another cycle of clofarabine. These three patients who received a not transplant intensification therapy are, so far, alive in complete remission. In eleven patients an allogeneic transplantation (with an unrelated or sibling donor) was performed as intensification therapy: to date, 8 of these 11 patients are alive in continuous complete remission after a median follow up of 6,5 months (2-36) while three died (two while in CR). Overall 11 (68%) of the 16 patients who entered into complete remission are alive and well. The new nucleoside analogue Clofarabine showed efficacy and synergism with cytarabine in treating AML. Even if 29/29 patients experienced grade IV haematological toxicity, infection rate was low and suitable considering the status of the disease. Extra haematological toxicity was acceptable and easily manageable. Considering the dismal outcome of these patients, we believe that intensification should be performed to allow a continuous complete remission. Indeed we were able to transplant 11/16 patients, eight of which maintained the complete remission after transplantation. Notably, 3 patients remained alive in complete remission out of a transplantation approach. It is not possible to draw any conclusion about the role, if any, of gemtuzumab ozogamicin. In summary, we showed that low dose clofarabine plus cytarabine because of efficacy and low extra-hematology toxicity could be a real bridge to allogeneic transplantation in these very poor risk pre-treated acute leukaemia patients. A longer follow up is awaited. Disclosures: Off Label Use: Clofarabine in refractpry/relapsed AML.

scholarly journals Efficacy and Safety of Decitabine in Combination with G-CSF, Low-Dose Cytarabine and Mitoxantrone in Pediatric Refractory and Relapse Acute Myeloid Leukemia and MDS-Raebt

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5175-5175
Author(s):  
Liyan Fan ◽  
Aili Chen ◽  
Yixin Hu ◽  
Peifang Xiao ◽  
Jun Lu ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Complete Remission ◽  
Induction Therapy ◽  
Myeloid Leukemia ◽  
Low Dose ◽  
Pediatric Patients ◽  
Efficacy And Safety ◽  
Hematopoietic Stem ◽  
Grade Ii ◽  
Acute Myeloid

Abstract Background: It is difficult for pediatric refractory and relapse acute myeloid leukemia (RR-AML) and MDS-RAEB/RAEBT patients to achieve complete remission (CR) and these patients develop recurrence and die of either disease progression or associated complications. The CAG regimen (cytarabine, aclarubicin and G-CSF) has been widely used in treating patients with MDS-EB and AML-MRC in Asia. Likely, Short term CAG derived regimens called low dose induction therapy, MAG regimen (Mitoxantrone for 3 doses, cytarabine and G-CSF for 10 days) also showed efficacy in De Novo AML. However, MAG regimen showed less efficacy in RR-AML and MDS-5(5q-). Purpose: To evaluate the clinical efficacy and safety of low-dose decitabine in combination with low-dose MAG regimen (D-MAG regimen) in the treatment of RR-AML and MDS-RAEB/RAEBT. Method A total of 17 patients with MDS-RAEB/RAEBT and RR-AML((2 cases of MDS-RAEB, 3 cases of MDS-RAEBT, 10 cases for refractory AML, and 2 cases for relapse AML) from June 2017 to April 2018 in our center were included in the retrospective study. All the patients were treated with decitabine of 20 mg/m2 for 5 days and followed by 10 days of MAG regimen (cytarabine of 10mg/m2 q12h for 10 days, mitoxantrone of 5 mg/ m2.d for 3 days, and G-CSF of 5μg/kg.d for 10 days),called D-CAG regimen. After two cycles of induction chemotherapy, the patients who obtained CR received consolidation chemotherapy or hematopoietic stem cell transplantation (HSCT). Results Among a total of 17 patients, the median age was the median age was 102 months (32-200 months) and 64.71 % of them were male. Characteristic features of these patients were illustrated in Table 1. Only 2 cases died of severe lung infection due to continuous agranulocytosis who had been received high dose induction therapy (Daunorubicin of 50mg/M2.d for 3 days, cytarabine of 100mg/m2 q12h for 10 days, and Etoposide of 150mg/m2.d for 5 days) for 2 cycles with poor physical condition before D-MAG. In the other 15 cases, 10 of them had complete remission (CR) after the first course of treatment, 4 cases were partial remission (PR), 1 case with a high blast cells at 25% indicated a poor response to D-MAG, and the effective rate was 93.3%. Among 4 children with PR, one case reached CR after the second courses of treatment. The most common adverse reactions in all children were hematological toxicity, grade III-IV. Liver damage was found in 2 cases, grade I and grade II respectively. There were 3 cases of oral side reactions, 1 case in grade I and 2 cases in grade II. The gastrointestinal reactions in all children were grade I - II. By July 2018, the median follow-up was 11 months (7-16months). Among 15 patients after D-MAG, 11 patients received HSCT. The twelve-month survival rate was 88.24% and the median leukemia-free survival (LFS) was 11 months. Conclusion The low-dose decitabine in combination with Low-dose MAG regimen improved CR rate for pediatric patients with MDS-RAEB and RR- AML, and is a promising treatment for pediatric patients with MDS/RR-AML. Disclosures No relevant conflicts of interest to declare.

scholarly journals New-Onset Diabetes Mellitus in Patients with Idiopathic Membranous Nephropathy Undergoing Tacrolimus and Low-Dose Corticosteroid Therapy

2019 ◽  
Vol 44 (6) ◽  
pp. 1352-1362 ◽  
Author(s):  
Lina Shao ◽  
Juan Jin ◽  
Binxian Ye ◽  
Baihui Xu ◽  
Yiwen Li ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Risk Factors ◽  
Risk Factor ◽  
Corticosteroid Therapy ◽  
Low Dose ◽  
Significant Difference ◽  
Dose Corticosteroid ◽  
New Onset Diabetes Mellitus ◽  
New Onset

Background: Idiopathic membranous nephropathy (IMN) is the most common cause of nephrotic syndrome in adults. Although various studies have demonstrated the efficacy of tacrolimus combined with corticosteroids for treating IMN, both tacrolimus and corticosteroids have been shown to be diabetogenic, particularly following organ transplantation. Furthermore, the frequency and risk factors for new-onset diabetes mellitus (NODM) in IMN patients treated with tacrolimus plus low-dose corticosteroids remain unclear. Objectives: To evaluate the incidence of NODM in IMN patients undergoing tacrolimus plus low-dose corticosteroid therapy and to confirm the risk factors for NODM development. Methods: This retrospective study recruited 72 eligible patients with biopsy-proven IMN from our center, between September 2013 and June 2018. All subjects were treated with tacrolimus plus low-dose corticosteroids for a minimum of 3 months. The primary outcome was NODM development during the follow-up period. The secondary outcome was complete or partial remission. Patients were divided into 2 groups: patients with NODM (NODM group) and those without NODM (No-NODM group). Demographic and clinical data at baseline and follow-up were assessed. Results: During follow-up, 31 of the 72 patients developed NODM (43.0%). The median time to occurrence was 3 months after treatment initiation. NODM patients were significantly older (median age 59 vs. 40 years) than No-NODM patients. Baseline fasting blood glucose levels were slightly higher in the NODM group; however, the difference was not significant (p = 0.07). Older age was an independent risk factor for NODM (OR 1.73 and 95% CI 1.20–2.47, p = 0.003). Overall kidney remission rates were 80.6%. There was no significant difference in remission rate between groups. There was a significant difference in development of pulmonary infection, which occurred in 7 NODM patients and only in 1 No-NODM patient (p = 0.018). IMN reoccurred in 5 NODM patients but only 1 No-NODM patient. Conclusions: Tacrolimus plus low-dose corticosteroid therapy was an efficient treatment for IMN; however, it was accompanied by increased NODM morbidity, which should be considered serious, due to the increased risk of life-threatening complications. Increasing age was a major risk factor for NODM in IMN patients treated with tacrolimus plus low-dose corticosteroid therapy.

scholarly journals Bone turnover biomarkers in COPD patients randomized to either a regular or shortened course of corticosteroids: a substudy of the randomized controlled CORTICO-COP trial

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Pradeesh Sivapalan ◽  
Niklas R. Jørgensen ◽  
Alexander G. Mathioudakis ◽  
Josefin Eklöf ◽  
Therese Lapperre ◽  
...  
Keyword(s):  
Corticosteroid Therapy ◽  
Dose Group ◽  
Low Dose ◽  
Corticosteroid Treatment ◽  
High Dose ◽  
Short Term ◽  
Copd Patients ◽  
Dose Corticosteroid

Abstract Background Long-term treatment with corticosteroids causes loss of bone density, but the effects of using short-term high-dose systemic-corticosteroid therapy to treat acute exacerbations of chronic obstructive pulmonary disease (AECOPD) are unclear. Our aim was to determine whether high-dose corticosteroid therapy affected bone turnover markers (BTMs) to a greater extent compared to low-dose corticosteroid therapy. Methods The CORTICO-COP trial (NCT02857842) showed that an eosinophil-guided corticosteroid intervention led to approximately 60% lower accumulated corticosteroid dose for hospitalized patients with AECOPD (low-dose group) compared with 5-day standard corticosteroid treatment (high-dose group). We compared the levels of BTMs C-terminal telopeptide of type 1 collagen (CTX) and procollagen type 1 N-terminal propeptide (P1NP) in 318 participants during AECOPD and at 1- and 3-month follow-up visits. Results CTX decreased and P1NP increased significantly over time in both treatment groups. There were no significant differences between the groups at 1- or 3-months follow-up for P1NP. A significant drop in CTX was seen at 3 months (down Δ24% from the baseline, p = 0.017) for the high dose group. Conclusion Short-term, high-dose systemic corticosteroid treatment caused a rapid suppression of biomarkers of bone resorption. Corticosteroids did not suppress biomarkers of bone formation, regardless of patients receiving low or high doses of corticosteroids. This therapy was, therefore, harmless in terms of bone safety, in our prospective series of COPD patients. Trial registration ClinicalTrials.gov Identifier: NCT02857842. Submitted August 2nd, 2016.

scholarly journals Serum Concentrations of Laminin-P1 in Thrombotic Microangiopathy

2000 ◽  
Vol 11 (3) ◽  
pp. 434-443
Author(s):  
ALFONS SEGARRA ◽  
RAFAEL SIMÓ ◽  
LLUIS MASMIQUEL ◽  
ROSA M. SEGURA ◽  
VICENS FONOLLOSA ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Basement Membrane ◽  
Renal Involvement ◽  
Serum Levels ◽  
Renal Outcome ◽  
Serum Concentrations ◽  
Healthy Control ◽  
Regular Hemodialysis

Abstract. Laminin is the main noncollagenous constituent of the basement membrane, and its serum levels could reflect the metabolic changes that occur in the basement membrane. Severe endothelial injury with thickening of basement membrane is a characteristic feature of thrombotic microangiopathy (TMA). With this background, the aim of the study was to investigate in a prospective way (1) the relationship among serum Lam-P1, the extent of renal histopathologic lesions, and the biochemical parameters commonly used as markers of TMA activity, and (2) the usefulness of serum Lam-P1 concentrations as a renal outcome prognostic index. To this end, 18 consecutive patients with active biopsy-proven TMA with renal involvement were studied. One hundred and twenty-one healthy control subjects, 20 patients with systemic scleroderma without renal involvement, and 35 patients with systemic lupus erythematosus (20 without nephropathy and 15 with diffuse proliferative type 4 lupus nephritis) were used as control groups. In addition, to analyze the influence of either renal failure or hemodialysis therapy on serum Lam-P1 levels, 91 patients on regular hemodialysis therapy and 81 patients with predialysis chronic renal failure of different etiologies were included in the study. Serum Lam-P1 was determined by RIA at admission, on days 10 and 30 of follow-up in all patients, and after 6 and 12 mo of follow-up in all surviving patients. Serum lactate dehydrogenase, haptoglobin, platelet count, hemoglobin, and serum creatinine were determined as markers of endothelial dysfunction and hemolysis. At admission, serum levels of Lam-P1 were significantly higher in patients with TMA than in healthy control subjects (3.39 ± 0.56 U/ml versus 1.40 ± 0.18 U/ml; P < 0.0001). In addition, patients with TMA had significantly higher serum Lam-P1 levels than the other groups included in the study. At the first control, Lam-P1 correlated with lactate dehydrogenase (P = 0.006) and hemoglobin (P = 0.002). During follow-up, platelet count and hemolysis indicators normalized in all patients, while serum Lam-P1 decreased only in patients with renal function recovery. In multivariate analysis, serum creatinine and Lam-P1 at day 10 were the only independent predictors of renal outcome (r2 = 0.94; P < 0.0001) and also correlated with indices of histopathologic damage (P < 0.001). Serum Lam-P1 normalized in all patients with chronic renal failure in the samples obtained at 6 and 12 mo of regular hemodialysis after solving active TMA, thus suggesting that histopathologic lesions, but not renal function itself, would be mainly responsible for the high Lam-P1 serum concentrations detected in TMA. In conclusion, serum Lam-P1 concentrations are increased in patients with active TMA. Furthermore, patients with poor renal outcome show a prolonged increase of serum Lam-P1 that is related to the extent of renal histologic lesions. Unlike the biochemical markers of hemolysis commonly used to assess TMA activity, the sequential determination of serum Lam-P1 provides valuable information about long-term renal prognosis in patients with TMA.

FC 039RENAL OUTCOME AFTER RITUXIMAB IN ADULT-ONSET IGA VASCULITIS AND CRESCENTIC IGA NEPHROPATHY: A MULTICENTRE STUDY

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Giorgio Trivioli ◽  
Alice Canzian ◽  
Federica Maritati ◽  
Roberta Fenoglio ◽  
Evangeline Pillebout ◽  
...  
Keyword(s):  
Renal Function ◽  
Renal Disease ◽  
Iga Nephropathy ◽  
Immunosuppressive Agents ◽  
Renal Involvement ◽  
Renal Outcome ◽  
Iga Vasculitis ◽  
Adult Onset ◽  
Free Survival

Abstract Background and Aims Glucocorticoids (GC) and/or immunosuppressive agents are the mainstay of therapy for adult-onset IgA Vasculitis (IgAV), but their efficacy is often partial while their toxicity is relevant. Recently, rituximab (RTX) has been reported as a safe and effective option but only few data on renal outcome are available.1 RTX has also been used in a few cases of crescentic IgA Nephropathy (cIgAN), an IgAN subset with vasculitic lesions and poor response to conventional immunosuppressive regimens.2 We present the results of a multicentre cohort of patients with IgAV and cIgAN treated with RTX. Method The databases of 16 consorted European centres were investigated to screen for patients with adult-onset, biopsy-proven IgAV and cIgAN (crescents in ≥25% glomeruli and rapid eGFR worsening at presentation), who received RTX as induction therapy. We selected patients with active renal manifestations at the time of RTX. Remission was defined as a Birmingham Vasculitis Activity Score (BVAS)=0 or &lt;5 if it was due to persistent proteinuria and relapse as an increase in BVAS requiring change in immunosuppressive therapy. Results We identified 38 patients with IgAV and 12 patients with cIgAN who received RTX and had active renal involvement at the time of treatment. The median age at onset was 40 years (interquartile range, IQR, 25-53) and more than two-thirds of patients were male (Table 1). The median follow-up after RTX was 41 months (IQR 18-60). Renal outcomes are reported in Table 2. At the time of treatment, 24 patients (48%) had eGFR ≥60 mL/min/1.73 m2. All had IgAV and their median BVAS was 17 (IQR 10-22). Furthermore, all had microhaematuria and proteinuria. Renal histology showed mesangial or focal endocapillary proliferation in 12/17 (71%) patients who underwent biopsy (class II-IIIA according to Pillebout3). Twenty patients (83%) achieved remission; after a median of 12 months (range 9-14), four experienced a minor relapse and one had a major relapse with significant renal disease progression. Renal function remained stable in all but two patients who developed end-stage renal disease (ESRD). Micro-haematuria subsided in 14/24 (58%) and median 24h proteinuria decreased from 1750 mg (IQR 865-3275) to 175 mg (IQR 100-800) at last follow-up (p=0.029). Of the 26 patients with eGFR &lt;60 mL/min/1.73 m2, 14 had IgAV and 12 had cIgAN. All were biopsied and 20 (77%) had diffuse endo/extra-capillary proliferation (classes IIIB-IV). Five patients required dialysis but recovered soon after treatment start. Remission was achieved by 16/26 (61%); eight (50%) subsequently relapsed and two (12%) reached ESRD. At last follow-up, eGFR was ≥60 mL/min/1.73 m2 in 8/26 (31%), 10/26 (48%) had stable renal function as compared to the time of RTX, while 8/26 (31%) had developed ESRD. Median 24h proteinuria decreased from 3400 mg (IQR 2150-6500) to 770 mg (177-1315) (p=0.016). Remission rate and ESRD-free survival were respectively 86% and 92% in patients with IgAV, while they were respectively 42% and 42% in cIgAN patients. Furthermore, 21/24 (87%) patients who received RTX alone or combined to glucocorticoids but not to immunosuppressive agents achieved remission and 22/24 (92%) were ESRD-free at last follow-up. Of the 26 patients receiving immunosuppressive agents, 17 (65%) obtained remission and 18 (69%) were ESRD-free at last assessment. Over the whole follow-up, only one patient reported a severe adverse effect related to RTX (pneumonia). Conclusion Renal involvement in adult-onset IgAV and cIgAN is frequently severe. RTX, combined or not with other immunosuppressive agents, may improve renal manifestations and is well tolerated. IgAV patients show higher remission rates and a longer ESRD-free survival as compared to cIgAN patients.

Pediatric onset lupus nephritis in western India—is it different from the rest of the country?

Lupus ◽  
2021 ◽  
pp. 096120332110450
Author(s):  
Aliza Mittal ◽  
Harshita Bamnawat ◽  
Aasma Nalwa ◽  
Vikarn Vishwajeet ◽  
Prawin Kumar ◽  
...  
Keyword(s):  
Renal Failure ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Care Center ◽  
Renal Involvement ◽  
Tertiary Care ◽  
Neurological Involvement ◽  
Western India ◽  
Pediatric Systemic Lupus Erythematosus

Aim To determine the clinicopathological characteristics and outcomes of children diagnosed with lupus nephritis in a tertiary hospital in western Rajasthan and compare it with the data available from other parts of India. Material and methods: A retrospective review of children presenting to a tertiary care center in western Rajasthan, India, with a diagnosis of pediatric Systemic Lupus Erythematosus (p SLE), between July 2017 and July 2020 was done. Comparisons of pediatric lupus in western India to other parts of country were done. Results 19 children with SLE with Renal involvement were enrolled and followed up. The median age at presentation was 15 years (IQR-16–9.5) (73% females). 8/19 (42%) children presented with AKI, of which 62% children presented as rapidly progressive renal failure. Six (37.5%) patients required dialysis at presentation. 84.21% of children were evaluated with renal biopsy, 16 biopsies were done in 19 children, among which class II, III, and IV lupus nephritis were reported in 21%,42%, and 35% respectively(4 crescentic). Antiphospholipid antibodies were positive in 8/15(53%), children which is much higher than a reported incidence of 30% in other Indian studies. Ten patients (52%) had neurological involvement, with seizures being the most common form of presentation (60%). Seven patients (36%) developed hepatitis. We noted many uncommon presentations in the small group like Autoimmune Pancreatitis, Mononeuritis multiplex, and peripheral digital gangrene. Cyclophosphamide was used in 10 out of 19 patients for inducing remission with class 3 and 4 nephritis and MMF in 8 children. 55% patients attained remission (after completing induction), of which 4 relapsed during the follow up. Four patients were lost to follow-up. A total of 27% patients died and 10% patients developed end stage renal failure. It was seen that those who died had more cardiac and neurological involvement at presentation, higher grade of proteinuria, lower GFR, and need for dialysis at admission. Conclusion: We found a more severe form of clinical manifestation in pediatric SLE patients at the time of the first presentation in the form of severe renal and extrarenal manifestation compared to other parts of the country.

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