Abstract
Background:
Currently, there are only few studies (mostly case reports or case series) on mycophenolate mofetil (MMF) in patients with systemic autoimmune myopathies (SAM). Therefore, the goal of the present study was to evaluate the safety and efficacy of MMF (monotherapy or coadjuvant drug) in a specific sample of patients with refractory SAM: dermatomyositis, polymyositis, anti-synthetase syndrome or clinically amyopathic dermatomyositis.
Methods:
A case series including 20 consecutive adult patients with refractory SAM from 2010 to 2016 was conducted. After the introduction of MMF, associated or not with other drugs, the patients were followed for 6 consecutive months.
Results:
In 17 out of 20 patients MMF was introduced without any intolerance. The clinical symptoms evaluated in these patients were muscular, cutaneous and/or pulmonary activity. During the 6-month follow-up, 11 out of 17 patients had clinical and laboratory activities response with MMF, allowing significant tapering of the prednisone median dose (15 vs. 5 mg/day, P=0.005). On the other hand, in three out of 20 patients; MMF was discontinued in less than two months, because of gastrointestinal intolerance. There were no cases of serious infection or death.
Conclusions:
MMF was relatively well-tolerated, safe and effective in patients with refractory SAM. Further studies are needed to confirm the data found.
Keywords:
Dermatomyositis; Drugs; Immunomodulator; Immunosuppressive; Myositis; Polymyositis
Background
Systemic autoimmune myopathies (SAM) are a heterogeneous group of rare systemic autoimmune diseases that result in progressive skeletal muscle weakness and disability [11. Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015;372:1734–47.–33. Nava A, Orozco-barocio G. Approach to the differential diagnosis of inflammatory myopathies. Rheumatol Clin. 2009;5:32–4.]. Depending on the demographic, clinical, laboratory, histological and disease evaluation, SAM can be classified into dermatomyositis (DM), polymyositis (PM), inclusion body myositis, or immune-mediated necrotizing myopathy, among others [22. Dalakas MC. Pathogenesis and therapies of immune-mediated myopathies. Autoimmun Rev. 2012;11:203–6.–44. Irazoque-Palazuelos F, Barragán-Navarro Y. Inflammatory myopathies: epidemiology, etiology and classification. Rheumatol Clin. 2009;5:2–5.].
There are no randomized controlled clinical trials and glucocorticoid has been used as the first-line drug in SAM [55. Aggarwal R, Oddis CV. Therapeutic advances in myositis. Curr Opin Rheumatol. 2012;24:635–41., 66. Distad BJ, Amato AA, Weiss MD. Inflammatory myopathies. Curr Treat Options Neurol. 2011;13:19–30.]. Various immunosuppressive or immunomodulatory drugs have been recommended as glucocorticoid-sparing agents, including methotrexate, azathioprine, cyclosporine, cyclophosphamide, tacrolimus and intravenous human immunoglobulin [55. Aggarwal R, Oddis CV. Therapeutic advances in myositis. Curr Opin Rheumatol. 2012;24:635–41.–77. Ernste FC, Reed AM. Idiopathic inflammatory myopathies: current trends in pathogenesis, clinical features, and up-to-date treatment recommendations. Mayo Clin Proc. 2013;88:83–105.]. Moreover, the rituximab, an anti-CD20 immunobiological drug, has been administered in refractory SAM cases [77. Ernste FC, Reed AM. Idiopathic inflammatory myopathies: current trends in pathogenesis, clinical features, and up-to-date treatment recommendations. Mayo Clin Proc. 2013;88:83–105., 88. Ytterberg SR. Treatment of refractory polymyositis and dermatomyositis. Curr Rheumatol Rep. 2006;8:167–73.].
Mycophenolate mofetil (MMF) is an agent that inhibits the mitosis and proliferation of T and B lymphocytes and has been successfully used to treat different autoimmune systemic diseases [99. Bandelier C, Guerne PA, Genevay S, Finckh A, Gabay C. Clinical experience with mycophenolate mofetil in systemic autoimmune conditions refractory to common immunosuppressant therapies. Swiss Med Wkly. 2009;139:41–6.]. However, only a few studies in the literature have investigated the use of MMF in adult patients with SAM [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9.–2020. Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.]. Furthermore, as limitations, the majority of these studies are case reports or case series [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9., 1212. Tausche AK, Meurer M. Mycophenolate mofetil for dermatomyositis. Dermatology. 2001;202:341–3., 1313. Gelber AC, Nousari HC, Wigley FM. Mycophenolate mofetil in the treatment of severe skin manifestations of dermatomyositis: a series of 4 cases. J Rheumatol. 2000;27:1542–5., 1515. Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res. 2010;62:1496–501.–1818. Parziale N, Kovacs SC, Thomas CB, Srinivasan J. Rituximab and mycophenolate combination therapy in refractory dermatomyositis with multiple autoimmune disorders. J Clin Neuromuscul Dis. 2011;13:63–7., 2020. Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.] and analyzed only SAM patients with pulmonary disease activity [1515. Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res. 2010;62:1496–501., 1919. Mira-Avendano IC, Parambil JG, Yadav R, Arrossi V, Xu M, Chapman JT, et al. A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis / dermatomyositis. Respir Med. 2013;107:890–6., 2020. Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.]. Those who used rituximab [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9.–1717. Danieli MG, Calcabrini L, Calabrese V, Marchetti A, Loqullo F, Gabrielli A. Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis. Autoimmun Rev. 2009;9:124–9., 1919. Mira-Avendano IC, Parambil JG, Yadav R, Arrossi V, Xu M, Chapman JT, et al. A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis / dermatomyositis. Respir Med. 2013;107:890–6., 2020. Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.] or anti-synthetase syndrome (ASS) patients [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9.–2020. Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.] have not been studied.
The aim of the present case series was to evaluate the safety and efficacy of MMF (monotherapy or coadjuvant drug) in refractory SAM (DM, PM, ASS or clinically amyopathic DM) as monotherapy or in combination of immunosuppressants.
Methods
This retrospective, case series included 21 consecutive adult patients with refractory SAM: classical DM or PM, according to Bohan and Peter's criteria [2121. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:344–7., 2222. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292:403–7.], clinically amyopathic DM, according to Gerami et al. [2323. Gerami P, Schope JM, McDonald L, Alling HW, Sontheimer RD. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006;54:597–613.], and ASS which was defined as myositis, arthritis, pulmonary disease, positive anti-synthetase antibody, with or without mechanic's hands, fever and/or Raynaud's phenomenon [2424. Mahler M, Miller FW, Fritzler MJ. Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review. Autoimmun Rev. 2014;13:367–71.].
Refractoriness was defined as primarily cutaneous (worsing heliotrope rash and/or Gottron's sign, new cutaneous lesions attributed to MAS), muscular (objective and progressive limb weakness), articular (arthritis) and/or pulmonary activity (progressive dyspnea), hampering glucocorticoid tapering and/or inadequate response to at least two immunosuppressive or immunomodulatory drugs at full-dose for a minimum period of three months, given sequentially or concomitantly [2525. Brandão M, Marinho A. Idiopathic inflammatory myopathies: Definition and management of refractory disease. Autoimmun Rev. 2011;10:720–4.].
To improve the homogeneity of the sample under study, only patients followed up at our outpatient clinic between 2010 and 2016 were included.
MMF treatment was defined as effective when the drug promoted over 50% improvement in the initial: cutaneous (evaluated clinically by the rheumatologists from Outgoing clinic); muscular (clinical muscle strength graded according to the Medical Research Council [2626. Medical Research Council: Aids to the investigation of peripheral nerve injuries. War Memorandun. No 7, 2. Ed. London: Her Majesty's Stationery Office, 1943.]) and/or laboratory parameters (serum creatine phosphokinase level - reference range: 24 - 173 IU/L - assayed by automated kinetic methods)]; articular (arthritis) or pulmonary (subjective dyspnea associated simultaneously with confirmed “ground-glass” on high-resolution chest computed tomography) activity. Comparisons of creatine phosphokinase level values at initial and after 6 months of MMF were considered as expected when variations ranged up to 20%. Moreover, glucocorticoid tapering of over 50% of initial dose was also considered evidence of efficacy of MMF.
All patients were followed for 6 consecutive months after MMF introduction and were examined at baseline and after 6 months by the same examiner.
Myositis overlap syndromes, neoplasia associated myositis, necrotizing myopathies, muscular dystrophy, inclusion body myositis, metabolic myopathies, irregular or doubt treatment adhesions were excluded.
Data were obtained from the ongoing electronic database protocol applied all patients with SAM at 1 - 6 month intervals entailing extensive clinical and laboratory evaluations, including the assessment relevant to this study.
Statistical analysis. The Kolmogorov-Smirnov test was used to evaluate the distribution of each parameter. The demographic and clinical features are expressed as the means ± standard deviations for the continuous variables or as frequencies and percentages for the categorical variables. The medians (25th −75th percentiles) were calculated for the continuous variables that were not normally distributed. Comparisons between the patients at initial and after 6 months of MMF were performed using Student's t-test or Wilcoxon test for continuous variables, and P < 0.05 was considered significant. All of the analyses were performed with the SPSS 15.0 statistics software (Chicago, USA).
Results
Twenty consecutive patients with refractory SAM treated with MMF were initially analyzed. In 7 patients, previous immunosuppressive drugs were exchanged for MMF (monotherapy), whereas in 13, MMF was associated with previous immunosuppressant (Table 1).
Patients #11 used rituximab 12 months before switch to MMF.
As an internal service protocol, the patients were not using antimalarials, except for one patient (#5).
In 17 out of 20 refractory MAS patients (11 DM, three PM, two ASS, one clinically amyopathic DM) (Table 1), MMF was introduced with good tolerance and with 100% of adhesion. The median dose of MMF was 2 g/day. This group comprised patients that were predominantly women, with a mean age of 46.2 ± 12.6 years and median disease duration of 2.0 years. All 17 patients used glucocorticoids (methylprednisolone or prednisone) and received previously a median of three immunosuppressive drugs (Table 1).
Of this group, 8 had muscle activity, three muscular and skin activities, three cutaneous activities, two pulmonary activities, one cutaneous and pulmonary activity and one had muscular, cutaneous and pulmonary activity. No cases had articular activity.
During the 6-month follow-up, prednisone median dose was significant tapering from 15.0 to 5.0 mg/day (P = 0.005). Moreover, the prednisone tapering was achieved in 14 out of 17 patients. However, glucocorticoid tapering of more than half occurred in 11 patients, all of whom had good clinical activity response using MMF.
As an additional analysis, the MAS patients with MMF as monotherapy (n = 6) were compare to those with MMF in combination therapy (n = 11). All clinical, laboratory, therapeutic and outcome parameters were comparable between both groups (P > 0.05).
In three out of 20 refractory female patients (one DM, one PM and one ASS) with cutaneous, articular and/or muscular activity, MMF was suspended in less than two month, because of gastrointestinal intolerance. The maximum dose of MMF in these patients was 1.5 g/day.
There were no cases of death or infection during the follow-up of the patients analyzed.
Discussion
This case series showed that MMF, as a monotherapy or coadjunt drug, is relatively safe and effective in patients with refractory SAM.
A strict exclusion in rare diseases criteria was employed in this study, however a sample of 20 consecutive patients with refractory SAM was analyzed based on previously standardized and parameterized data. The protocol was performed at the same service adopting the same standardization of reports, thereby reducing inter-examiner variability. Only patients with refractoriness were included.
MMF has been used in several systemic autoimmune diseases, such as systemic sclerosis, rheumatoid arthritis, Sjögre's syndrome, systemic lupus erythematosus [1616. Saketkoo LA, Espinoza LR. Experience of mycophenolate mofetil in 10 patients with autoimmune-related interstitial lung disease demonstrates promising effects. Am j Med Sci. 2009;337:329–35., 2727. Appel GB, Gerald B, Radhakrishnan J, Ginzler EM. Use of mycophenolate mofetil in autoimmune and renal diseases. Transplantation. 2005;80:S265–71.–2929. Cunha GF, Souza FH, Levy-Neto M, Shinjo SK. Chloroquine diphosphate: a risk factor for herpes zoster in patients with dermatomyositis / polymyositis. Clinics. 2013;68:621–7.]. However, there are few studies in the literature investigating the use of MMF in adult patients with SAM [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9.–2020. Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.].
Most studies are case reports or case series and MMF was found to promote significant clinical and laboratory improvement in patients with SAM [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9., 1212. Tausche AK, Meurer M. Mycophenolate mofetil for dermatomyositis. Dermatology. 2001;202:341–3.–1616. Saketkoo LA, Espinoza LR. Experience of mycophenolate mofetil in 10 patients with autoimmune-related interstitial lung disease demonstrates promising effects. Am j Med Sci. 2009;337:329–35., 1919. Mira-Avendano IC, Parambil JG, Yadav R, Arrossi V, Xu M, Chapman JT, et al. A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis / dermatomyositis. Respir Med. 2013;107:890–6., 2020. Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.].
According to the study by Majithia and Harisdangkul [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9.], 6 out of 7 refractory SAM had marked improvement, with good tolerance, in active myositis using MMF. This response rate was higher than ours, however in a group with less severity and in previous use of a smaller number of immunosuppressive drugs.
In another study [1313. Gelber AC, Nousari HC, Wigley FM. Mycophenolate mofetil in the treatment of severe skin manifestations of dermatomyositis: a series of 4 cases. J Rheumatol. 2000;27:1542–5.], MMF was effective for controlling cutaneous activity in all four patients with SAM analyzed, also resulting in glucocorticoid tapering. In 10 out of 12 patients with recalcitrant DM, Edge et al. [1414. Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol. 2006;142:65–9.] observed an improvement in muscular and cutaneous activity after four weeks of treatment with MMF.
Probably we found a smaller rate of success because of all patients of our sample had refractory and severe disease.
The heterogeneity of response evaluation in myopathies in the literature is present. Better criteria have been established [3030. Bruce B, Fries JF. The Stanford Health Assessment Questionnaire: dimensions and practical applications. Health Qual Life Outcomes. 2003;1:20.–3333. Sultan SM, Allen E, Oddis CV, Kiely P, Cooper RG, Lundberg IE, et al. Reliability and validity of the myositis disease activity assessment tool. Arthritis Rheum. 2008;58:3593–9.], but in relation to DM, for example, there is still a difficulty in assessing improvement, especially in those with little muscle involvement. The response assessment parameter of the present study was based mainly on the clinical criteria.
Previous study showed that antimalarial could predispose patients with DM/PM to developing herpes zoster, particularly women and DM patients [2929. Cunha GF, Souza FH, Levy-Neto M, Shinjo SK. Chloroquine diphosphate: a risk factor for herpes zoster in patients with dermatomyositis / polymyositis. Clinics. 2013;68:621–7.]. Therefore, as an internal service protocol, our patients were not using antimalarials (except for one patient) at the time of this study.
Facing the previous refractoriness, in two thirds of the patients, the MMF was introduced as a coadjuvant in the present study. However, during follow-up, there was no difference between this group and those who used MMF as monotherapy for the response parameters analyzed.
In the present study, most frequent side effects of MMF were associated with the gastrointestinal tract (nausea, vomiting, abdominal pain and/or diarrhea). Intolerance was observed in three out of the 20 patients in the present analysis, comparable to findings of other studies [1212. Tausche AK, Meurer M. Mycophenolate mofetil for dermatomyositis. Dermatology. 2001;202:341–3.–1414. Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol. 2006;142:65–9.].
Akin to the present study, some investigations have also shown that MMF is safe in patients with SAM [1010. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9., 1414. Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol. 2006;142:65–9., 1616. Saketkoo LA, Espinoza LR. Experience of mycophenolate mofetil in 10 patients with autoimmune-related interstitial lung disease demonstrates promising effects. Am j Med Sci. 2009;337:329–35.]. There were no cases of infection or death events in our sample. By contrast, Rowin et al. [1111. Rowin J, Amato AA, Deisher N, Cursio J, Meriggioli MN. Mycophenolate mofetil in dermatomyositis: is it safe? Neurology. 2006;66:1245–7.] reported that three out of their 10 DM patients developed opportunistic infections with MMF (pulmonary infections: Blastomycosis, Mycobacterium xenopi, legionella).
Limitations of this study include the short follow-up of 6 months. In addition, possible inclusion of more severe cases of the disease due to the characteristics of our tertiary care centre should also be considered. Finally, as this is a review of retrospective cases, tools such as Manual Muscle Testing (MMT)-8 [3131. Rider LG, Koziol D, Giannini EH, Jain MS, Smith MR, Whitney-Mahoney K, et al. Validation of manual muscle testing and a subset of eight muscles for adult and juvenile idiopathic inflammatory myopathies. Arthritis Care Research (Hoboken). 2010;62:465–72.], 2016 European League Against Rheumatism / American College of Rheumatology (EULAR/ACR) response criteria [3434. Rider LG, Ruperto N, Pistorio A, Erman B, Bayat N, Lachenbruch PA, et al. International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects. Rheumatology (Oxford). 2017;56:1884–93.] were not used and pulmonary involvement was not analyzed with pulmonary function test (at baseline and 6 months of MMF) and high-resolution chest computed tomography (6 months of MMF).
Conclusions
MMF was relatively well-tolerated, safe and effective in patients with refractory SAM, at least in the short follow-up of 6 consecutive months. Further studies are needed to confirm the data found in the present study.
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Availability of data and materialsPlease contact author for data requests.
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Ethics approval and consent to participateThis study was approved by the local Ethics Committee (HCFMUSP, CAPPesq, Number 0039/10) and all participant signed the informed consent form.
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Consent for publicationNot applicable.
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Publisher's NoteSpringer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Abbreviations
- AM: Antimalarials
- ASS: Anti-synthetase syndrome
- AZA: Azathioprine
- C: Cutaneous
- CADM: Clinically amyopathic dermatomyositis
- CP: Cyclosporine
- CPK: Creatine phosphokinase
- CYC: Cyclophosphamide
- DM: Dermatomyositis
- EULAR/ACR: European League Against Rheumatism / American College of Rheumatology
- F: Female
- IVIg: Intravenous human immunoglobulin
- LFN: Leflunomide
- M: Male
- MMF: Mycophenolate mofetil
- MMT: Manual Muscle Testing
- MP: Methylprednisolone pulse therapy
- Mu: Muscular
- P: Pulmonary
- PM: Polymyositis
- Pred: Prednisone
- RTX: Rituximab
- SAM: Systemic autoimmune myopathies
- Tac: Tacrolymus
References
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1Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015;372:1734–47.
-
2Dalakas MC. Pathogenesis and therapies of immune-mediated myopathies. Autoimmun Rev. 2012;11:203–6.
-
3Nava A, Orozco-barocio G. Approach to the differential diagnosis of inflammatory myopathies. Rheumatol Clin. 2009;5:32–4.
-
4Irazoque-Palazuelos F, Barragán-Navarro Y. Inflammatory myopathies: epidemiology, etiology and classification. Rheumatol Clin. 2009;5:2–5.
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5Aggarwal R, Oddis CV. Therapeutic advances in myositis. Curr Opin Rheumatol. 2012;24:635–41.
-
6Distad BJ, Amato AA, Weiss MD. Inflammatory myopathies. Curr Treat Options Neurol. 2011;13:19–30.
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7Ernste FC, Reed AM. Idiopathic inflammatory myopathies: current trends in pathogenesis, clinical features, and up-to-date treatment recommendations. Mayo Clin Proc. 2013;88:83–105.
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8Ytterberg SR. Treatment of refractory polymyositis and dermatomyositis. Curr Rheumatol Rep. 2006;8:167–73.
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9Bandelier C, Guerne PA, Genevay S, Finckh A, Gabay C. Clinical experience with mycophenolate mofetil in systemic autoimmune conditions refractory to common immunosuppressant therapies. Swiss Med Wkly. 2009;139:41–6.
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10Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44: 386–9.
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11Rowin J, Amato AA, Deisher N, Cursio J, Meriggioli MN. Mycophenolate mofetil in dermatomyositis: is it safe? Neurology. 2006;66:1245–7.
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12Tausche AK, Meurer M. Mycophenolate mofetil for dermatomyositis. Dermatology. 2001;202:341–3.
-
13Gelber AC, Nousari HC, Wigley FM. Mycophenolate mofetil in the treatment of severe skin manifestations of dermatomyositis: a series of 4 cases. J Rheumatol. 2000;27:1542–5.
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14Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol. 2006;142:65–9.
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15Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res. 2010;62:1496–501.
-
16Saketkoo LA, Espinoza LR. Experience of mycophenolate mofetil in 10 patients with autoimmune-related interstitial lung disease demonstrates promising effects. Am j Med Sci. 2009;337:329–35.
-
17Danieli MG, Calcabrini L, Calabrese V, Marchetti A, Loqullo F, Gabrielli A. Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis. Autoimmun Rev. 2009;9:124–9.
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19Mira-Avendano IC, Parambil JG, Yadav R, Arrossi V, Xu M, Chapman JT, et al. A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis / dermatomyositis. Respir Med. 2013;107:890–6.
-
20Tsuchiya H, Tsuno H, Inoue M, Takahashi Y, Yamashita H, Kaneko H, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol. 2014;24:694–6.
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-
23Gerami P, Schope JM, McDonald L, Alling HW, Sontheimer RD. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006;54:597–613.
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24Mahler M, Miller FW, Fritzler MJ. Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review. Autoimmun Rev. 2014;13:367–71.
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29Cunha GF, Souza FH, Levy-Neto M, Shinjo SK. Chloroquine diphosphate: a risk factor for herpes zoster in patients with dermatomyositis / polymyositis. Clinics. 2013;68:621–7.
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30Bruce B, Fries JF. The Stanford Health Assessment Questionnaire: dimensions and practical applications. Health Qual Life Outcomes. 2003;1:20.
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31Rider LG, Koziol D, Giannini EH, Jain MS, Smith MR, Whitney-Mahoney K, et al. Validation of manual muscle testing and a subset of eight muscles for adult and juvenile idiopathic inflammatory myopathies. Arthritis Care Research (Hoboken). 2010;62:465–72.
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32Rider LG, Feldman BM, Perez MD, Rennebohm RM, Lindsley CB, Zemel LS, et al. Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies: I. Physician, parent, and patient global assessments. Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Arthritis Rheum. 1997;40:1976–83.
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33Sultan SM, Allen E, Oddis CV, Kiely P, Cooper RG, Lundberg IE, et al. Reliability and validity of the myositis disease activity assessment tool. Arthritis Rheum. 2008;58:3593–9.
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34Rider LG, Ruperto N, Pistorio A, Erman B, Bayat N, Lachenbruch PA, et al. International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects. Rheumatology (Oxford). 2017;56:1884–93.
Publication Dates
-
Publication in this collection
29 July 2019 -
Date of issue
2018
History
-
Received
20 Mar 2018 -
Accepted
07 Oct 2018 -
Published
22 Oct 2018