International Journal of Rheumatic Diseases 2013; 16: 556–560
ORIGINAL ARTICLE
Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade? Shiva PRASAD, Ramnath MISRA, Vikas AGARWAL, Able LAWRENCE and Amita AGGARWAL Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
Abstract Introduction: Juvenile dermatomyositis (JDM) is a rare multisystem disorder of childhood primarily involving the skeletal muscles and skin. Patients and methods: The case records of patients with JDM seen at our centre in the last 10 years were reviewed and data on clinical presentation, management, outcome and complications were retrieved. Results: Eighteen patients (nine boys) were diagnosed as JDM with median age at presentation of 12.5 years, duration of illness of 9.25 months and follow-up duration of 24 months. At presentation, rash was seen in all patients, 17 had muscle weakness, fever in 11 and arthritis in six. Gottron’s lesions and heliotrope rash were seen in 14 and 11 patients, respectively. Calcinosis was seen in five patients and lipoatrophy in two patients. Four patients had dysphagia, one each had dilated cardiomyopathy and respiratory failure. Electromyograph was abnormal in 15 patients and antinuclear antibodies were positive in nine patients. Prednisolone and methotrexate were used in 17 patients. Other disease-modifying anti-rheumatic drugs used were hydroxychloroquine, azathioprine, cyclophosphamide and cyclosporine. Sixteen patients achieved remission. Five patients had pyogenic infections and one patient died of this. In addition two patients had tuberculosis. Conclusion: Compared to our experience in the previous decade we saw more girls, used methotrexate upfront but the median duration of illness and prevalence of calcinosis (30%) was the same, suggesting that we need to improve awareness about JDM among paediatricians for early referral. Key words: calcinosis, juvenile dermatomyositis, lipoatrophy, outcome, paediatric rheumatology.
INTRODUCTION Juvenile dermatomyositis (JDM) is a rare, vasculopathic autoimmune disease of childhood, characterized by proximal symmetric muscle weakness, in addition to characteristic skin rash. It constitutes about 2.0–3.3% of pediatric rheumatology cases.1,2 Due to lack of effective treatment, outcome was poor prior to 1960 with mortality in one-third of patients and one-third developing
Correspondence: Dr Amita Aggarwal, Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. Email: [email protected], [email protected]
serious disabilities.3 With refinement in the management of JDM in developed countries over the last three decades, and a dramatic reduction in the mortality of JDM, attention has switched from survival to continued disease activity, muscle strength, physical function, cumulative organ damage, and health-related quality of life (HRQOL).4 However, in developing countries, the majority of the patients are diagnosed late, so complications such as calcinosis, lipoatrophy, contractures and muscle damage are still common. Our previous data had shown a high prevalence of these complications.5 With a view to see if there is any change over the last decade we analyzed the cases seen in this decade and compared it with previously published data of the last decade.5
© 2013 The Authors International Journal of Rheumatic Diseases © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd
JDM at tertiary care hospitals
PATIENTS AND METHODS All patients diagnosed as JDM (Bohan and Peter criteria6) at the Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, between 2002 and 2011, were included. Retrospective review of case records was done to collect data regarding demography, clinical features at presentation, laboratory data, outcome and complications.
RESULTS Eighteen patients were diagnosed with JDM during this period, nine were boys and had median disease duration (onset of first symptom to diagnosis) of 9.25 months. Clinical and demographic profiles are given in Table 1. One patient presented with fever, rash and calcinosis without weakness and was diagnosed as amyopathic JDM. (This patient did not fulfil the Bohan and Peter criteria). Calcinosis (Figs 1 and 2) was present in five (27.7%) patients. Lipoatrophy was detected in two (11.1%) patients. Most of the patients had elevated muscle enzymes. Electromyograph (EMG) was performed on 15 patients and all were abnormal with myopathic pattern and a few had evidence of fibrillation and increased insertional activity. Muscle biopsy was performed on one patient and was suggestive of dermatomyositis. Magnetic resonance imaging (MRI) of the muscle was performed on two patients and both revealed muscle inflammation. Antibodies to nuclear antigens (ANA) were positive in nine (performed on 17) patients and antibodies to extractable nuclear antigen were positive in four (performed on 13) patients. Two patients had anti-Ro antibodies, two had anti-Jo1 antibodies and one had anti-nRNP antibody positivity. None of our patients had overlap syndrome. One patient who presented with proximal muscle weakness with pharyngeal involvement, heliotrope and Gottron’s rash and myopathic pattern on EMG was diagnosed as having malignancyrelated JDM. This patient had lymphoma diagnosed on lymph node biopsy and was transferred to the hematology unit for further management. Thus data on 17 patients’ outcomes was analyzed. Seventeen patients received prednisolone 1 mg/kg/ day. Oral methotrexate was the most frequent steroidsparing disease-modifying drug used in 17 patients. No patient received parenteral methotrexate. Intravenous methyl-prednisolone boluses for 3 days were used in
International Journal of Rheumatic Diseases 2013; 16: 556–560
Figure 1 Photograph of the abdomen showing multiple subcutaneous nodules.
Table 1 Clinical and demographic profile of patients Parameter Median age at presentation (range) Median duration of illness at presentation (range) Boys:girls Muscle weakness Gottron’s rash Heliotrope rash Mechanic’s hand Arthritis/arthralgia Raynaud’s phenomenon Respiratory failure Dysphagia Dysphonia Calcinosis Lipoatrophy Dilated cardiomyopathy
No. 12.5 years (2.5–16) 9.25 months (0.5–120) 9:9 17 14 10 1 6 1 1 4 2 5 2 1
patients with respiratory muscle weakness, dysphagia or cardiomyopathy. Intravenous cyclophosphamide was used2 in two patients who were refractory to conventional treatment. The other disease-modifying drugs used were hydroxychloroquinine in four patients with persistent skin rash, azathioprine in three, and cyclosporine in one patient with relapse or refractory disease. Tocilizumab was used in one child as she had persistent fever and had not responded to methotrexate, cyclophosphamide or cyclosporine. Alendronate and pamidronate were used in two patients for calcinosis. One patient who had not responded to alendronate
557
S. Prasad et al.
was shifted to pamidronate. One patient had stabilization of calcinosis, whereas in another it was too early to assess. Intravenous immunoglobulins and rituximab are beyond the reach of majority of our patients as health care in India is private funded. The median follow-up was 24 months. Follow-up duration was 96 months in one patient, 56 months in one, 36 months in five, 24 months in four, 23 months in one and 12 months in two and less than a year in three patients. The major reasons for loss of follow-up were financial and social situations. The other reason was complete remission of the disease. Of 16 patients who went into remission, five patients relapsed. Monophasic illness was observed in 11, polyphasic in five and chronic phase in one patient. All but one patient were surviving at the last visit. The child with a chronic course with persistent fever and rash had no response to steroids, methotrexate, cyclophosphamide or cyclosporine. She was later treated with tocilizumab with which her systemic symptoms improved but she died due to staphylcoccus sepsis. Pyogenic infections were the most common therapyrelated complications. Pneumonia occurred in one, pyogenic abscess in two, urinary tract infection in two, and sepsis and skin ulcer in one patient each. Tuberculosis (TB) occurred in two (11.1%) patients.
DISCUSSION There were 18 patients with equal numbers of boys and girls. The median duration of illness and median age at presentation were 9.25 months and 12.5 years, respectively. All patients except one achieved remission, but
calcinosis and lipoatropy were present in 30% and 11.1%, respectively. In contrast to our previous data where we had male preponderance, there was no gender bias in this study.5 This is in contrast to the Western literature where the disease is slightly more frequent in girls than boys with a sex ratio of approximately 1.6:1.0. Asian studies from other centres from India and Japan report a male preponderance (Table 2).7,8 This change in a decade could reflect changes in social norms where female children are receiving better care. The median duration of illness has not changed, suggesting that we still have a poor referral system and JDM being a rare disease is not recognized early enough. This could be related to inadequate exposure to pediatric rheumatological diseases in undergraduate and postgraduate curricula. Further, in the early phase, fever and rash are often thought to be due to infection. In a country where infections form a major part of pediatric practice, muscle weakness usually goes unnoticed in a sick child as the child stays confined to bed. Thus all children with non-resolving fever and rash should be evaluated for other causes such as lupus and JDM. The approach to diagnosis of JDM has changed in the last decade with less use of EMG and muscle biopsy as both are invasive procedures. Instead, use of short tau inversion recovery (STIR) and fat-suppressed MRI is gaining popularity to assess inflammation in muscles.9 Whole body MRI is being evaluated as a tool to screen the degree of muscle inflammation.10 In the previous study we performed six muscle biopsies but in the present series only one was done, again supporting the above change in practice. Magnetic resonance imaging was not used in the previous study but was performed on two patients in the present series. Calcinosis occurred in nearly 30% of patients. This is similar to previous data from our centre and also Table 2 Comparison between different Indian studies Parameters
Figure 2 Radiograph of the pelvis showing subcutaneous calcinosis.
558
No. of patients M:F Median age at presentation (years) Median duration of illness (months) Calcinosis (%) Lipoatrophy (%)
Prasad (present study) 2012
Chowdhary5 2002
Singh9 2006
18 1:1 12.5 (2.5–16)
19 1.7:1 12
33 1.7:1 8.7
9.25 (0.5–120)
12
14
27.0 11.1
26.3 NA
27.3 30.3
International Journal of Rheumatic Diseases 2013; 16: 556–560
JDM at tertiary care hospitals
from other centres from India.5,11 This may be the consequence of delay in diagnosis resulting in delay in treatment. Persistent disease activity has been linked to calcinosis.12,13 One patient was referred for unremitting fever, rash and calcinosis without any weakness. Lipoatrophy was rare with only two patients in this series and none in the last series.5 The incidence of lipoatrophy here is less than in another study from India.11 Lack of lipoatrophy in our previous series could be related to lack of awareness about this rare complication at that time.5 Calcinosis and lipodystrophy, are associated with a greater cumulative duration of active disease.12–14 In one of the studies from India it was found that 65% of patients with JDM had loss of subcutaneous fat on quantification compared with 40% on physical appearance alone. They also had hypertriglyceridemia and insulin-resistant diabetes mellitus.15 Persistent presence of rash at 3 months after diagnosis and nailfold abnormalities has been found to predict a longer time to remission.16 Chronic course, either polycyclic or persistent, is consistently associated with poorer functional ability and HRQOL in the physical domain.17 Most patients achieved complete remission with drugs. Juvenile dermatomyositis, although rare, has good response to corticosteroids. In our previous study 42% of patients achieved complete remission and partial remission was observed in 42.1% of cases.5 Better response in this cohort could be related to upfront use of methotrexate. Seventeen patients were started on methotrexate upfront, whereas in the previous study only 13 patients had received methotrexate.5 Two patients were in the chronic phase in the previous study5 while one patient was in the chronic phase in the present study. Tuberculosis was detected in 11.1% of patients as compared to 21% of patients in the previous study.5 The incidence of TB has varied from 5.0 to 13.7% in lupus patients and is associated with higher mortality.18–20 Isoniazid (INH) prophylaxis has been found to be efficacious in acquired autoimmuno-deficiency syndrome (AIDS), hematological malignancies and renal transplants; however, data is sparse in systemic lupus erythematosus (SLE)/JDM.21,22 INH prophylaxis reduced the incidence of TB from 11% to 2% without any significant side effects in patients with SLE in western India.23 Thus it may be worth considering INH prophylaxis in children with JDM. However, INH prophylaxis is controversial in a high disease burden country like India. We did not use INH prophylaxis in our hospital as it can increase the risk of drug resistance.
International Journal of Rheumatic Diseases 2013; 16: 556–560
CONCLUSIONS Juvenile dermatomyositis is a rare disease even at a tertiary care hospital, but if diagnosed early and treated adequately it can have good outcome. Almost one-third of patients develop calcinosis in India, which probably is related to delay in treatment. Our data over two decades show that we need to increase awareness in practising physicians, undergraduates and postgraduates about JDM diagnosis and treatment, in order to prevent long-term complications.
REFERENCES 1 Feldman BM, Rider LG, Reed AM, Pachman LM (2008) Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet 371, 2201–12. 2 Seth V, Kabra SK, Semwal OP, Jain Y (1996) Juvenile dermatomyositis. Indian J Pediatr 63, 375–9. 3 Huber A, Feldman BM (2005) Long-term outcomes in juvenile dermatomyositis: how did we get here and where are we going? Cur Rheumatol Rep 7, 441–6. 4 Huber AM, Lang B, LeBlanc CM et al. (2000) Medium-and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum 43, 541–9. 5 Chowdhary V, Wakhlu A, Agarwal A, Misra R (2002) Outcome in Juvenile Dermatomyositis. Indian Pediatr 39, 931–5. 6 Bohan A, Peter JB (1975) Polymyositis and dermatomyositis. N Engl J Med 292, 344–407. 7 Singh S, Kumar L, Ravi Shankar K (1997) Juvenile dermatomyositis in north India. Indian Pediatr 34, 193–8. 8 Hiketa T, Matsumoto Y, Ohashi M, Sasaki R (1992) Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis. J Dermatol 19, 470–6. 9 Davis WR, Halls JE, Offiah AC, Pilkington C, Owens CM, Rosendahl K (2011) Assessment of active inflammation in juvenile dermatomyositis: a novel magnetic resonance imaging-based scoring system. Rheumatology (Oxford) 50, 2237–44. 10 Tzaribachev N, Well C, Schedel J, Horger M (2009) Whole-body MRI: a helpful diagnostic tool for juvenile dermatomyositis case report and review of the literature. Rheumatol Int 29, 1511–14. 11 Singh S, Bansal A (2006) Twelve years experience of juvenile dermatomyositis in North India. Rheumatol Int 26, 510–15. 12 Bowyer SL, Blane CE, Sullivan DB, Cassidy JT (1983) Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. J Pediatr 103(88), 2–8.
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13 Fisler RE, Liang MG, Fuhlbrigge RC, Yalcindag A, Sundel RP (2002) Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis. J Am Acad Dermatol 47, 505–11. 14 Tabarki B, Ponsot G, Prieur AM, Tardieu M (1998) Childhood dermatomyositis: clinical course of 36 patients treated with low doses of corticosteroids. Eur J PaediatrNeurol 2, 205–11. 15 Verma S, Singh S, Bhalla AK, Khullar M (2006) Study of subcutaneous fat in children with juvenile dermatomyositis. Arthritis Rheum 55, 564–8. 16 Stringer E, Singh-Grewal D, Feldman BM (2008) Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features. Arthritis Rheum 58, 3585–92. 17 Ravelli A, Trail L, Ferrari C et al. (2010) Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients. Arthritis Care Res 62, 63–72.
560
18 Lliopoulos AG, Tsokos GC (1996) Immunopathogenesis and spectrum of infections in systemic lupus erythematosus. Semin Arthritis Rheum 25, 318–36. 19 Balakrishnan C, Mangat G, Mittal G, Joshi VR (1998) Tuberculosis in patients with systemic lupus erythematosus. J Assoc Physicians India 46, 682–3. 20 Feng PH, Tan TH (1982) Tuberculosis in patients with systemic lupus erythematosus. Ann Rheum Dis 41, 11–14. 21 Moriuchi Y, Kamihara S, Satoh T et al. (1991) Infection prophylaxis in patients with haematological malignancies Successful prophylaxis of tuberculosis with isoniazid. Rinsho Ketsueki (Japanese J Clin Hematol) 32, 199–204. 22 Yildiz A, Sukru Sever M, Turkmen A et al. (1998) Tuberculosis after renal transplantation: experience of one Turkish centre. Nephrol Dial Transplant 13, 1872–5. 23 Gaitonde S, Pathan E, Sule A, Mittal G, Joshi VR (2002) Efficacy of isoniazid prophylaxis in patients with systemic lupus erythematosus receiving long term steroid treatment. Ann Rheum Dis 61, 251–3.
International Journal of Rheumatic Diseases 2013; 16: 556–560
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ORIGINAL ARTICLE
Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade? Shiva PRASAD, Ramnath MISRA, Vikas AGARWAL, Able LAWRENCE and Amita AGGARWAL Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
Abstract Introduction: Juvenile dermatomyositis (JDM) is a rare multisystem disorder of childhood primarily involving the skeletal muscles and skin. Patients and methods: The case records of patients with JDM seen at our centre in the last 10 years were reviewed and data on clinical presentation, management, outcome and complications were retrieved. Results: Eighteen patients (nine boys) were diagnosed as JDM with median age at presentation of 12.5 years, duration of illness of 9.25 months and follow-up duration of 24 months. At presentation, rash was seen in all patients, 17 had muscle weakness, fever in 11 and arthritis in six. Gottron’s lesions and heliotrope rash were seen in 14 and 11 patients, respectively. Calcinosis was seen in five patients and lipoatrophy in two patients. Four patients had dysphagia, one each had dilated cardiomyopathy and respiratory failure. Electromyograph was abnormal in 15 patients and antinuclear antibodies were positive in nine patients. Prednisolone and methotrexate were used in 17 patients. Other disease-modifying anti-rheumatic drugs used were hydroxychloroquine, azathioprine, cyclophosphamide and cyclosporine. Sixteen patients achieved remission. Five patients had pyogenic infections and one patient died of this. In addition two patients had tuberculosis. Conclusion: Compared to our experience in the previous decade we saw more girls, used methotrexate upfront but the median duration of illness and prevalence of calcinosis (30%) was the same, suggesting that we need to improve awareness about JDM among paediatricians for early referral. Key words: calcinosis, juvenile dermatomyositis, lipoatrophy, outcome, paediatric rheumatology.
INTRODUCTION Juvenile dermatomyositis (JDM) is a rare, vasculopathic autoimmune disease of childhood, characterized by proximal symmetric muscle weakness, in addition to characteristic skin rash. It constitutes about 2.0–3.3% of pediatric rheumatology cases.1,2 Due to lack of effective treatment, outcome was poor prior to 1960 with mortality in one-third of patients and one-third developing
Correspondence: Dr Amita Aggarwal, Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. Email: [email protected], [email protected]
serious disabilities.3 With refinement in the management of JDM in developed countries over the last three decades, and a dramatic reduction in the mortality of JDM, attention has switched from survival to continued disease activity, muscle strength, physical function, cumulative organ damage, and health-related quality of life (HRQOL).4 However, in developing countries, the majority of the patients are diagnosed late, so complications such as calcinosis, lipoatrophy, contractures and muscle damage are still common. Our previous data had shown a high prevalence of these complications.5 With a view to see if there is any change over the last decade we analyzed the cases seen in this decade and compared it with previously published data of the last decade.5
© 2013 The Authors International Journal of Rheumatic Diseases © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd
JDM at tertiary care hospitals
PATIENTS AND METHODS All patients diagnosed as JDM (Bohan and Peter criteria6) at the Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, between 2002 and 2011, were included. Retrospective review of case records was done to collect data regarding demography, clinical features at presentation, laboratory data, outcome and complications.
RESULTS Eighteen patients were diagnosed with JDM during this period, nine were boys and had median disease duration (onset of first symptom to diagnosis) of 9.25 months. Clinical and demographic profiles are given in Table 1. One patient presented with fever, rash and calcinosis without weakness and was diagnosed as amyopathic JDM. (This patient did not fulfil the Bohan and Peter criteria). Calcinosis (Figs 1 and 2) was present in five (27.7%) patients. Lipoatrophy was detected in two (11.1%) patients. Most of the patients had elevated muscle enzymes. Electromyograph (EMG) was performed on 15 patients and all were abnormal with myopathic pattern and a few had evidence of fibrillation and increased insertional activity. Muscle biopsy was performed on one patient and was suggestive of dermatomyositis. Magnetic resonance imaging (MRI) of the muscle was performed on two patients and both revealed muscle inflammation. Antibodies to nuclear antigens (ANA) were positive in nine (performed on 17) patients and antibodies to extractable nuclear antigen were positive in four (performed on 13) patients. Two patients had anti-Ro antibodies, two had anti-Jo1 antibodies and one had anti-nRNP antibody positivity. None of our patients had overlap syndrome. One patient who presented with proximal muscle weakness with pharyngeal involvement, heliotrope and Gottron’s rash and myopathic pattern on EMG was diagnosed as having malignancyrelated JDM. This patient had lymphoma diagnosed on lymph node biopsy and was transferred to the hematology unit for further management. Thus data on 17 patients’ outcomes was analyzed. Seventeen patients received prednisolone 1 mg/kg/ day. Oral methotrexate was the most frequent steroidsparing disease-modifying drug used in 17 patients. No patient received parenteral methotrexate. Intravenous methyl-prednisolone boluses for 3 days were used in
International Journal of Rheumatic Diseases 2013; 16: 556–560
Figure 1 Photograph of the abdomen showing multiple subcutaneous nodules.
Table 1 Clinical and demographic profile of patients Parameter Median age at presentation (range) Median duration of illness at presentation (range) Boys:girls Muscle weakness Gottron’s rash Heliotrope rash Mechanic’s hand Arthritis/arthralgia Raynaud’s phenomenon Respiratory failure Dysphagia Dysphonia Calcinosis Lipoatrophy Dilated cardiomyopathy
No. 12.5 years (2.5–16) 9.25 months (0.5–120) 9:9 17 14 10 1 6 1 1 4 2 5 2 1
patients with respiratory muscle weakness, dysphagia or cardiomyopathy. Intravenous cyclophosphamide was used2 in two patients who were refractory to conventional treatment. The other disease-modifying drugs used were hydroxychloroquinine in four patients with persistent skin rash, azathioprine in three, and cyclosporine in one patient with relapse or refractory disease. Tocilizumab was used in one child as she had persistent fever and had not responded to methotrexate, cyclophosphamide or cyclosporine. Alendronate and pamidronate were used in two patients for calcinosis. One patient who had not responded to alendronate
557
S. Prasad et al.
was shifted to pamidronate. One patient had stabilization of calcinosis, whereas in another it was too early to assess. Intravenous immunoglobulins and rituximab are beyond the reach of majority of our patients as health care in India is private funded. The median follow-up was 24 months. Follow-up duration was 96 months in one patient, 56 months in one, 36 months in five, 24 months in four, 23 months in one and 12 months in two and less than a year in three patients. The major reasons for loss of follow-up were financial and social situations. The other reason was complete remission of the disease. Of 16 patients who went into remission, five patients relapsed. Monophasic illness was observed in 11, polyphasic in five and chronic phase in one patient. All but one patient were surviving at the last visit. The child with a chronic course with persistent fever and rash had no response to steroids, methotrexate, cyclophosphamide or cyclosporine. She was later treated with tocilizumab with which her systemic symptoms improved but she died due to staphylcoccus sepsis. Pyogenic infections were the most common therapyrelated complications. Pneumonia occurred in one, pyogenic abscess in two, urinary tract infection in two, and sepsis and skin ulcer in one patient each. Tuberculosis (TB) occurred in two (11.1%) patients.
DISCUSSION There were 18 patients with equal numbers of boys and girls. The median duration of illness and median age at presentation were 9.25 months and 12.5 years, respectively. All patients except one achieved remission, but
calcinosis and lipoatropy were present in 30% and 11.1%, respectively. In contrast to our previous data where we had male preponderance, there was no gender bias in this study.5 This is in contrast to the Western literature where the disease is slightly more frequent in girls than boys with a sex ratio of approximately 1.6:1.0. Asian studies from other centres from India and Japan report a male preponderance (Table 2).7,8 This change in a decade could reflect changes in social norms where female children are receiving better care. The median duration of illness has not changed, suggesting that we still have a poor referral system and JDM being a rare disease is not recognized early enough. This could be related to inadequate exposure to pediatric rheumatological diseases in undergraduate and postgraduate curricula. Further, in the early phase, fever and rash are often thought to be due to infection. In a country where infections form a major part of pediatric practice, muscle weakness usually goes unnoticed in a sick child as the child stays confined to bed. Thus all children with non-resolving fever and rash should be evaluated for other causes such as lupus and JDM. The approach to diagnosis of JDM has changed in the last decade with less use of EMG and muscle biopsy as both are invasive procedures. Instead, use of short tau inversion recovery (STIR) and fat-suppressed MRI is gaining popularity to assess inflammation in muscles.9 Whole body MRI is being evaluated as a tool to screen the degree of muscle inflammation.10 In the previous study we performed six muscle biopsies but in the present series only one was done, again supporting the above change in practice. Magnetic resonance imaging was not used in the previous study but was performed on two patients in the present series. Calcinosis occurred in nearly 30% of patients. This is similar to previous data from our centre and also Table 2 Comparison between different Indian studies Parameters
Figure 2 Radiograph of the pelvis showing subcutaneous calcinosis.
558
No. of patients M:F Median age at presentation (years) Median duration of illness (months) Calcinosis (%) Lipoatrophy (%)
Prasad (present study) 2012
Chowdhary5 2002
Singh9 2006
18 1:1 12.5 (2.5–16)
19 1.7:1 12
33 1.7:1 8.7
9.25 (0.5–120)
12
14
27.0 11.1
26.3 NA
27.3 30.3
International Journal of Rheumatic Diseases 2013; 16: 556–560
JDM at tertiary care hospitals
from other centres from India.5,11 This may be the consequence of delay in diagnosis resulting in delay in treatment. Persistent disease activity has been linked to calcinosis.12,13 One patient was referred for unremitting fever, rash and calcinosis without any weakness. Lipoatrophy was rare with only two patients in this series and none in the last series.5 The incidence of lipoatrophy here is less than in another study from India.11 Lack of lipoatrophy in our previous series could be related to lack of awareness about this rare complication at that time.5 Calcinosis and lipodystrophy, are associated with a greater cumulative duration of active disease.12–14 In one of the studies from India it was found that 65% of patients with JDM had loss of subcutaneous fat on quantification compared with 40% on physical appearance alone. They also had hypertriglyceridemia and insulin-resistant diabetes mellitus.15 Persistent presence of rash at 3 months after diagnosis and nailfold abnormalities has been found to predict a longer time to remission.16 Chronic course, either polycyclic or persistent, is consistently associated with poorer functional ability and HRQOL in the physical domain.17 Most patients achieved complete remission with drugs. Juvenile dermatomyositis, although rare, has good response to corticosteroids. In our previous study 42% of patients achieved complete remission and partial remission was observed in 42.1% of cases.5 Better response in this cohort could be related to upfront use of methotrexate. Seventeen patients were started on methotrexate upfront, whereas in the previous study only 13 patients had received methotrexate.5 Two patients were in the chronic phase in the previous study5 while one patient was in the chronic phase in the present study. Tuberculosis was detected in 11.1% of patients as compared to 21% of patients in the previous study.5 The incidence of TB has varied from 5.0 to 13.7% in lupus patients and is associated with higher mortality.18–20 Isoniazid (INH) prophylaxis has been found to be efficacious in acquired autoimmuno-deficiency syndrome (AIDS), hematological malignancies and renal transplants; however, data is sparse in systemic lupus erythematosus (SLE)/JDM.21,22 INH prophylaxis reduced the incidence of TB from 11% to 2% without any significant side effects in patients with SLE in western India.23 Thus it may be worth considering INH prophylaxis in children with JDM. However, INH prophylaxis is controversial in a high disease burden country like India. We did not use INH prophylaxis in our hospital as it can increase the risk of drug resistance.
International Journal of Rheumatic Diseases 2013; 16: 556–560
CONCLUSIONS Juvenile dermatomyositis is a rare disease even at a tertiary care hospital, but if diagnosed early and treated adequately it can have good outcome. Almost one-third of patients develop calcinosis in India, which probably is related to delay in treatment. Our data over two decades show that we need to increase awareness in practising physicians, undergraduates and postgraduates about JDM diagnosis and treatment, in order to prevent long-term complications.
REFERENCES 1 Feldman BM, Rider LG, Reed AM, Pachman LM (2008) Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet 371, 2201–12. 2 Seth V, Kabra SK, Semwal OP, Jain Y (1996) Juvenile dermatomyositis. Indian J Pediatr 63, 375–9. 3 Huber A, Feldman BM (2005) Long-term outcomes in juvenile dermatomyositis: how did we get here and where are we going? Cur Rheumatol Rep 7, 441–6. 4 Huber AM, Lang B, LeBlanc CM et al. (2000) Medium-and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum 43, 541–9. 5 Chowdhary V, Wakhlu A, Agarwal A, Misra R (2002) Outcome in Juvenile Dermatomyositis. Indian Pediatr 39, 931–5. 6 Bohan A, Peter JB (1975) Polymyositis and dermatomyositis. N Engl J Med 292, 344–407. 7 Singh S, Kumar L, Ravi Shankar K (1997) Juvenile dermatomyositis in north India. Indian Pediatr 34, 193–8. 8 Hiketa T, Matsumoto Y, Ohashi M, Sasaki R (1992) Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis. J Dermatol 19, 470–6. 9 Davis WR, Halls JE, Offiah AC, Pilkington C, Owens CM, Rosendahl K (2011) Assessment of active inflammation in juvenile dermatomyositis: a novel magnetic resonance imaging-based scoring system. Rheumatology (Oxford) 50, 2237–44. 10 Tzaribachev N, Well C, Schedel J, Horger M (2009) Whole-body MRI: a helpful diagnostic tool for juvenile dermatomyositis case report and review of the literature. Rheumatol Int 29, 1511–14. 11 Singh S, Bansal A (2006) Twelve years experience of juvenile dermatomyositis in North India. Rheumatol Int 26, 510–15. 12 Bowyer SL, Blane CE, Sullivan DB, Cassidy JT (1983) Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. J Pediatr 103(88), 2–8.
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International Journal of Rheumatic Diseases 2013; 16: 556–560
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