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   2012| July  | Volume 7 | Issue 5  
    Online since July 6, 2016

 
 
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REVIEW ARTICLES
Macrophage activation syndrome
Bianca Lattanzi, Sergio Davi, Silvia Rosina, Nicoletta Solari, Stefano Lanni, Giulia Bracciolini, Alberto Martini, Angelo Ravelli
July 2012, 7(5):27-35
DOI:10.1016/S0973-3698(12)60026-0  
Macrophage activation syndrome (MAS) is a serious, potentially life-threatening complication of rheumatic disorders, which is seen most commonly in systemic juvenile idiopathic arthritis (sJIA). It is characterised clinically by unremitting high fever, pancytopaenia, hepatosplenomegaly, hepatic dysfunction, encephalopathy, coagulation abnormalities and sharply increased levels of ferritin. The pathognomonic feature of the syndrome is seen on bone marrow examina- tion, which frequently, though not always, reveals numerous morphologically benign macrophages exhibiting hae- mophagocytic activity. Macrophage activation syndrome is overt in 10% of children with sJIA but occurs subclinically in another 30-40%. Because MAS can follow a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are essential. However, it is difficult to distinguish sJIA disease flare, infectious complications or medication side effects from MAS. A multinational collaborative effort aimed to develop diagnostic criteria for MAS in sJIA is under way. Although, the pathogenesis of MAS is unclear, the hallmark of the syn- drome is an uncontrolled activation and proliferation of T lymphocytes and macrophages, leading to massive hyper- secretion of pro-inflammatory cytokines. Mutations in cytolytic pathway genes are increasingly being recognised in children who develop MAS as part of sJIA. Recently, a mouse model of MAS dependent on repeated stimulation through toll-like receptors was developed. The first-line therapy of MAS complicating sJIA is based on the parenteral administration of high doses of corticosteroids, with or without cyclosporine A. There is increasing evidence that bio- logical therapies, particularly interleukin-1 inhibitors, represent a valuable adjunct to corticosteroids and cyclosporine A in treating MAS complicating sJIA.
[ABSTRACT]   Full text not available  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]
  988 129 4
Juvenile dermatomyositis: A review of clinical features and management
S Habibi, AV Ramanan
July 2012, 7(5):80-86
DOI:10.1016/S0973-3698(12)60032-6  
Juvenile dermatomyositis is the most common of the idiopathic inflammatory myositis of childhood. It is characterised by a pathognomic skin rash along with proximal muscle weakness. It may also affect organs other than the muscle and skin. Calcinosis and lipodystrophy are complications of long-standing untreated or poorly treated disease, asso- ciated with chronic morbidity. A variety of environmental and genetic risk factors are implicated in its pathogenesis. Up-regulation of genes by type-I interferon appears central. Corticosteroids along with methotrexate are the mainstay of therapy. Disease outcome is in general good.
[ABSTRACT]   Full text not available  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]
  915 140 2
Henoch-Schonlein purpura: An update
Nutan Kamath, Suchetha Rao
July 2012, 7(5):92-98
DOI:10.1016/S0973-3698(12)60034-X  
Henoch-Schonlein purpura (HSP), the commonest childhood vasculitis, is characterised by non-thrombocytopaenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. The peak incidence is between the ages of 4 years and 8 years with a male preponderance. Though the diagnosis is usually clinical, a tissue biopsy revealing leucocytoclastic vasculitis is helpful when the presentation is atypical. Renal involvement in the form of an immune complex glomerulonephritis is the most serious long-term complication. The aetiopathogenesis, classifica- tion, clinical features, relevant Indian data, and a stepwise management approach with corticosteroids and immuno- suppressive agents per the renal histology are discussed.
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  824 131 3
Juvenile idiopathic arthritis: Classification, clinical features, and management
Sujata Sawhney
July 2012, 7(5):11-21
DOI:10.1016/S0973-3698(12)60024-7  
This paper discusses the current classification system for juvenile arthritis, describes the clinical features and diffi- culties encountered with the International League of Associations of Rheumatologists classification in the clinic and discusses the management options for children with juvenile idiopathic arthritis (JIA). In a resource-constrained country such as ours, the best practice is early diagnosis, rapid referral to centres of excellence, aggressive treatment from the outset, avoidance of deformities and treat to a target of remission for all children with appropriate changes to therapy if the goal of inactive disease is not achieved. With these simple principals, many children with JIA may be treated without biologics, a group of drugs that is clearly beyond the reach of a majority of Indian children with JIA.
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  666 142 2
Management of lupus nephritis
Ashima Gulati, Arvind Bagga
July 2012, 7(5):69-79
DOI:10.1016/S0973-3698(12)60031-4  
Systemic lupus erythematosus is a multisystemic, autoimmune disorder, with varied clinical and immunological man- ifestations. Though the predominant onset is in young adulthood especially in females, childhood-onset, with a much broader spectrum of clinical presentations, has been reported with incidence rates ranging from 0.36 to 2.5 per 100,000. Diverse immune system aberrations, both inherited and acquired, contribute to the organ damage. Lupus nephritis is seen in a high proportion of childhood-onset disease and is an important contributor for both short-term and long-term morbidity. Various immunosuppressive regimes with a common goal of achieving and maintaining renal remission have been tested mainly in the adult population. The management of childhood-onset lupus nephritis based on the current available evidence is reviewed and the most appropriate management approach for this group of patients is discussed.
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  677 100 -
ABSTRACTS
Future directions in paediatric rheumatology
Balu H Athreya
July 2012, 7(5):3-6
DOI:10.1016/S0973-3698(12)60022-3  
Paediatric rheumatology has come of age. In a country as large as India with its large childhood population with rheumatic diseases, there is a great need to develop training programmes in paediatric rheumatology. Fundamental research is needed to study the incidence, prevalence and variations in clinical features of common rheumatic dis- eases in the Indian population. Newer modalities of therapy need to be developed to suit local needs and conditions. Tools to evaluate outcomes need to be developed to suit the cultural conditions of the land. Resources have to be developed to provide continuity of care to these children with chronic diseases in remote villages. All of this requires planning, leadership, sustained effort and collaboration between patients and physicians and between private and public agencies.
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  636 76 1
REVIEW ARTICLES
Long-term outcome in juvenile idiopathic arthritis
Amita Aggarwal, Sajjan Shenoy, Ranjan Gupta
July 2012, 7(5):22-26
DOI:10.1016/S0973-3698(12)60025-9  
Juvenile idiopathic arthritis is a chronic arthritis of childhood that continues in adulthood in about two-third children. In the past, it was associated with significant morbidity as reflected by physical disability, joint deformities, growth abnormalities, joint loss necessitating joint replacement at a young age along with decline in the psychosocial qual- ity of life. In addition, most children took more time to complete education though they achieved their full potential in the end. In recent years, with better and early control of disease activity with the use of intra-articular steroids, methotrexate and biologics, the outcome has improved significantly with a hope that in future these children will have a near normal life.
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  619 89 1
Acute rheumatic fever
Vijay Viswanathan
July 2012, 7(5):36-43
DOI:10.1016/S0973-3698(12)60027-2  
Acute rheumatic fever (ARF) and its sequelae rheumatic heart disease (RHD) are systemic inflammatory conditions following group A beta haemolytic streptococcal (GABHS) pharyngitis. The condition primarily affects children between 5 years and 15 years of age mainly in developing countries and in indigenous populations of developed countries. Pathogenesis of the disease remains an enigma and specific treatment is not available; hence there is a lot of emphasis on prevention of initial and recurrent attacks. With protean manifestations, a high index of suspi- cion along with precise interpretation of clinical criteria (modified Jones criteria) is needed for the diagnosis. Clinical guidelines on the management of this condition with reference to the Indian scenario have been formulated. Anti- inflammatory agents such as aspirin and steroids are the mainstay of symptomatic treatment of rheumatic fever. Primary prevention of ARF is accomplished by proper identification and adequate antibiotic treatment of GABHS tonsillopharyngitis. The most effective approach for control of ARF and RHD is secondary prophylaxis.
[ABSTRACT]   Full text not available  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]
  621 82 2
Spectrum of paediatric rheumatologic disease: The Mumbai experience
Raju P Khubchandani, Rachana P Hasija
July 2012, 7(5):7-10
DOI:10.1016/S0973-3698(12)60023-5  
The concept of paediatric rheumatology clinics is relatively new to India. With the specialty still in its formative years, referrals are still delayed and children usually present after having visited multiple facilities. Our centre, which has been one of the "early birds" in setting up a paediatric rheumatology clinic in a tertiary hospital in Mumbai, reports on the spectrum of cases seen since its inception in 2003 and thereby provides a framework for those wanting to open such centres in India or for that matter in countries where paediatric rheumatology is coming of age.
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  605 95 -
Takayasu's arteritis: Paediatric perspective
Sathish Kumar
July 2012, 7(5):99-105
DOI:10.1016/S0973-3698(12)60035-1  
Takayasu's arteritis (TA) is a rare chronic granulomatous vasculitis of the aorta and its branches and is associated with considerable morbidity and premature mortality. Initial symptoms and signs are non-specific. High index of suspicion is needed to make the correct diagnosis. The clinical manifestations in paediatric patients are less specific than in adults: in children, the disease presents with fever, arthralgias and hypertension. In this review, we detail the epidemiology, pathophysiology, clinical features, imaging characteristics, and various treatment options for TA in children.
[ABSTRACT]   Full text not available  [PDF]  [Mobile Full text]  [EPub]
  572 49 -
Rheumatic manifestations of primary immunodeficiencies in children
Manjari Agarwal, Mehul Jariwala
July 2012, 7(5):52-56
DOI:10.1016/S0973-3698(12)60029-6  
Primary immunodeficiency disorders (PIDs) usually present with a variety of infections due to a defect in the body's defence mechanisms which can affect both the adaptive and the innate immune systems. The infections can involve bones and joints. Beside infections, autoimmune diseases are the second most common feature of PID. Arthritis is the most common rheumatological manifestation of PIDs. Consider PID in a child who presents with recurrent septic arthritis, arthritis associated with increased susceptibility to infection, autoimmune disease involving multiple organs or systemic lupus erythematosus with negative antinuclear antibody (ANA).
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  575 34 2
Arthropathy in haematological disorders in children
Deepti Suri, Sapna Oberoi
July 2012, 7(5):44-51
DOI:10.1016/S0973-3698(12)60028-4  
Haematological disorders, both benign and malignant, may present with osteoarticular complaints. Overlapping rheumatological symptoms can easily mislead the treating physician away from the underlying haematological ill- ness. Suspecting and diagnosing the primary blood disorder is crucial in these patients. In this article, we review the spectrum of haematological disorders commonly associated with arthropathy, their clinical presentation and man- agement. We will further highlight the common diagnostic pitfalls that will help the treating clinician in managing such children.
[ABSTRACT]   Full text not available  [PDF]  [Mobile Full text]  [EPub]
  537 53 -
Arthralgia and limb pains: Pseudo-rheumatoid genetic disorders
Shubha Phadke
July 2012, 7(5):57-68
DOI:10.1016/S0973-3698(12)60030-2  
Limb and joint pains can be the manifestation of many infectious, autoimmune or genetic disorders of the bones and joints. In addition, it may also be the manifestation of many genetic or non-genetic disorders of other systems. The monogenic disorders manifesting with joint and limb pains need to be suspected when the rheumatologist concludes that the disorder does not fit the pattern of a specific rheumatic disease. The accurate diagnosis is important as specific treatments are available for some genetic disorders. Diagnosis of genetic disorders also has implications for the family and indicates the need for genetic counselling. In this article, the various situations where a genetic cause for limb and joint pains need to be suspected are discussed. In addition, there is a brief review of some monogenic disorders which often present to the rheumatologist as joint disease.
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  537 45 -
Kawasaki disease-A preventable cause of cardiac morbidity
Anju Gupta, Surjit Singh
July 2012, 7(5):87-91
DOI:10.1016/S0973-3698(12)60033-8  
Kawasaki disease is a common acute childhood vasculitis that can leave behind serious coronary sequelae. Though, it continues to be commonest in Japan, from where it was first described, it has been now described from all over the world. No specific aetiologic agent is known. Similarly, no single clinical feature or laboratory investigation is diagnostic. Intravenous immunoglobulin is the gold standard of care and if given within 10 days of onset, is associ- ated with a significantly reduced risk of coronary aneurysms. Despite appropriate care, 3% children still develop coronary artery abnormalities and require long-term aspirin therapy with or without anticoagulants. All children should be on long-term cardiac care because of risk of premature atherosclerosis and dyslipidaemia.
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  532 29 1
Glucocorticoid use in children: The problems and solutions
Tushar R Godbole, Preeti Dabadghao
July 2012, 7(5):112-116
DOI:10.1016/S0973-3698(12)60037-5  
Despite their deleterious effects, glucocorticoids (GCs) are commonly used in various disorders in the paediatric age group. Children on GC therapy need close monitoring for growth failure and other side effects. This article gives an overview about the problems associated with GC use and the present status on their solutions.
[ABSTRACT]   Full text not available  [PDF]  [Mobile Full text]  [EPub]
  514 47 -
Laboratory tests and the paediatric rheumatologist: What does he need to know?
Sita Naik
July 2012, 7(5):106-111
DOI:10.1016/S0973-3698(12)60036-3  
Rheumatic diseases form an important group of diseases in children, since early and correct diagnosis and man- agement are important for their growth and development. Diagnosis of these conditions is highly supported buy laboratory investigations. This article provides an overview of the laboratory investigations that are useful in the management of rheumatic diseases in general with special emphasis on their interpretation in children.
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  514 43 -
ABSTRACTS
Paediatric rheumatology: What has changed in last 10 years?
Ross S Petty
July 2012, 7(5):1-2
DOI:10.1016/S0973-3698(12)60021-1  
Full text not available  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]
  463 71 1
FROM THE EDITORíS DESK
From the Editor's desk
Amita Aggarwal, Sujata Sawhney
July 2012, 7(5):0-0
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  449 60 -
REVIEW ARTICLES
Important publication of Indian Pediatric Rheumatology (original articles only) for past 5 years (2006-2012)
Varun Dhir
July 2012, 7(5):119-122
DOI:10.1016/S0973-3698(12)60039-9  
Full text not available  [PDF]  [Mobile Full text]  [EPub]
  384 32 -