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1.
Clin Exp Rheumatol ; 40(4): 841-848, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35383555

RESUMO

OBJECTIVES: High-dose glucocorticoids for remission-induction of ANCA-associated vasculitis are recommended and commonly used in adults, but recent studies suggest lower glucocorticoid doses can reduce toxicity without reducing efficacy. No paediatric-specific data exists to inform optimal glucocorticoid dosing in paediatric ANCA-associated vasculitis (pAAV). Our objectives were to describe glucocorticoid use in pAAV-related renal disease, and to explore associations between glucocorticoid dose, baseline patient characteristics and 12-month outcomes. METHODS: Youth <18 years with pAAV, biopsy-confirmed pauci-immune glomerulonephritis and 12-month follow-up data were included from an international paediatric vasculitis registry. Presenting features and 12-month outcomes (eGFR, glucocorticoid-related adverse effects), were compared between patients receiving no, low-moderate (≤90mg/kg) and high (>90mg/kg) cumulative intravenous methylprednisolone (IVMP), and low (<0.5mg/kg/day prednisone equivalent), moderate (0.5-1.5mg/kg/day) and high (>1.5mg/kg/day) starting doses of oral glucocorticoids. RESULTS: Among 131 patients (101 granulomatosis with polyangiitis, 30 microscopic polyangiitis), 27 (21%) received no IVMP, 64 (49%) low-moderate and 29 (22%) high-dose IVMP, while 9 (7%) received low, 75 (57%) moderate and 47 (36%) high initial doses of oral glucocorticoids. Renal failure at diagnosis (p=0.022) and plasmapheresis use (p=0.0001) were associated with high-dose IVMP. Rates of glucocorticoid-related adverse effects ranged from 15-31% across dose levels, and glucocorticoid dosing did not associate with 12-month outcomes. CONCLUSIONS: Glucocorticoid dosing for pAAV-related renal disease was highly variable, and rates of adverse effects were high across all dosing groups. A significant proportion of patients received oral glucocorticoid or IVMP doses that were discordant with current adult guidelines. Higher glucocorticoid doses did not associate with improved outcomes.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Poliangiite Microscópica , Adolescente , Adulto , Anticorpos Anticitoplasma de Neutrófilos , Criança , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Masculino , Indução de Remissão , Rituximab/uso terapêutico
2.
Int J Rheum Dis ; 27(6): e15243, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38925615

RESUMO

INTRODUCTION: Mixed connective tissue disease (MCTD) is a rare entity in children. There is a paucity of studies on juvenile-onset MCTD (jMCTD) worldwide especially from Southeast Asia. OBJECTIVES: To describe clinical and laboratory features of jMCTD diagnosed at pediatric rheumatology centers across India. METHODS: A predesigned detailed case proforma in an excel format was prepared and was sent to all the Pediatric Rheumatology centers in India. Eleven centers provided the clinical and laboratory data of their jMCTD patients, which was then compiled and analyzed in detail. RESULTS: Thirty-one jMCTD patients from 11 centers were included in the study. Our cohort had 27 females and four male patients over 12 months (August 2021 to July 2022). The median age at presentation was 12 years (range 5-18 years) and the median duration of symptoms was 24 months at diagnosis (range 2-96 months). The common features included arthritis (90%), malar rash (70.9%), and Raynaud's phenomenon (70.9%). At a mean follow-up of 43 months (range 1-168 months), 45% of them were in remission. There were two deaths reported, due to macrophage activation syndrome and sepsis respectively. CONCLUSION: We present the largest multicenter experience on jMCTD from the Indian subcontinent. The study's findings serve as a crucial stepping stone toward unraveling the complexities of jMCTD and improving patient care and management strategies.


Assuntos
Doença Mista do Tecido Conjuntivo , Humanos , Criança , Masculino , Feminino , Doença Mista do Tecido Conjuntivo/diagnóstico , Doença Mista do Tecido Conjuntivo/terapia , Doença Mista do Tecido Conjuntivo/epidemiologia , Índia/epidemiologia , Adolescente , Pré-Escolar , Resultado do Tratamento , Idade de Início , Imunossupressores/uso terapêutico , Antirreumáticos/uso terapêutico , Estudos Retrospectivos , Fatores de Tempo , Indução de Remissão
3.
Neurol India ; 70(1): 300-303, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35263900

RESUMO

Background: Juvenile dermatomyositis (JDM) is one of the commonest forms of inflammatory myositis in childhood. Objective: The objective study was to study the clinical characteristics and course of JDM patients. Material and Methods: Retrospective analysis of the charts of 25 JDM patients admitted to two hospitals in Bangalore from March 2011 to July 2017. Results: The mean age at onset of disease was 7.74 ± 3.74 years. The male to female ratio was 1.5:1. All patients had skin rashes typical of JDM and 24/25 had demonstrable muscle weakness. Six patients were either lost to follow-up or died. Of the remaining 19 patients, 11 (57.9%) had a monocyclic course, 5 (26.3%) patients had a chronic continuous course, and 3 (15.8%) patients had a polycyclic course. Conclusions: JDM though rare should always be considered in the differential diagnosis in any child with skin rash and muscle pains and weakness. When diagnosed early and treated appropriately, sustained remission without medications is possible in a good proportion of patients.


Assuntos
Dermatomiosite , Miosite , Criança , Pré-Escolar , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Feminino , Humanos , Índia , Masculino , Debilidade Muscular/etiologia , Estudos Retrospectivos
4.
Indian J Orthop ; 52(6): 672-677, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30532310

RESUMO

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare auto-inflammatory disease of the bone. It tends to be multifocal and usually the symptoms tend to run for months and years before diagnosis is usually made. The objective of our study was to understand the clinical presentation and short-term response to treatment of CRMO patients. MATERIALS AND METHODS: A retrospective analysis of patients diagnosed with CRMO between 2011 and 2016 was done. Case records of these were retrospectively reviewed for clinical features, investigations and treatment received. RESULTS: Six patients were diagnosed with CRMO. The median age of onset and time to diagnosis from onset of symptoms was 8 and 3.5 years respectively. Lower limb bones were the most commonly involved. CONCLUSIONS: There is significant delay in diagnosis of CRMO and this could be because of a lack of awareness of this condition amongst clinicians. Our case series with only male affection is rather unique as compared to other case series reported in medical literature which tend to have more female predilection. Pain with or without swelling was the most common symptom. Most of patients responded to combination therapy.

6.
Artigo | IMSEAR | ID: sea-204512

RESUMO

Background: Polyarteritis nodosa (PAN) is a primary systemic necrotizing vasculitis predominantly targeting medium-sized arteries defined as the main visceral arteries and their branches. The objective of this study was to analyze the epidemiology, clinico-pathological and point outcome of PAN in children at a tertiary care centre in Karnataka.Methods: This prospective observational study was conducted among eighteen children diagnosed with PAN from December 2015 to May 2017.' All the children fulfilling EULAR/PRES/PRINTO classification criteria for childhood PAN were included in this study. Clinico-pathological profile of these patients was studied and documented.Results: This study had shown that there are two peaks of age at onset of symptoms at 5 to 7 years and from 11 to 13 years. No sex predilection was noted. Most children present with nonspecific symptoms like myalgia (94%), fever (84%), weight loss (50%) and joint pain (50%). Skin manifestations were seen in all the children. Neurological involvement (61%) in the form of mononeuritis multiplex (38.8%), hypertensive encephalopathy (11%) and stroke (11%) was observed. Four children (22%) presented with pain abdomen while two boys had testicular pain. Only one child had renal manifestation in the form of glomerulonephritis. Most children had neutrophilic leukocytosis, elevated ESR and thrombocytosis. Skin biopsy was performed in ten children and out of these, 9 reports were suggestive of PAN. Angiography was done in four children revealing multiple aneurysms or stenosis in the systemic arteries. All the children received corticosteroids. Cyclophosphamide was used as an induction agent in about 50% of the patients. Corticosteroid treatment alone was sufficient in only one patient. Steroid sparing agent like azathioprine (38%), mycophenolate mofetil (33%) and hydroxychloroquine (5%) were used with low dose steroids as maintenance therapy. Seven children (39%) achieved remission off drugs while 8 children are still on treatment, one child relapsed and three children were lost to follow up.Conclusions: Childhood vasculitis leads to considerable delay in diagnosis the initiation of treatment. High index of suspicion and early referral is required in all cases of vasculitis to reduce morbidity and mortality.

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