Corticosteroids in Myositis and Scleroderma

Postolova, Anna; Chen, Jennifer K.; Chung, Lorinda

doi:10.1016/j.rdc.2015.08.011

Cited by 13 publications

(8 citation statements)

References 55 publications

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“…Some DMARDs are widely used; however, no drug has been found to have an unequivocal benefit. 23,[24][25][26] We initially use oral corticosteroids in combination with DMARDs (MTX, mycophenolate-mofetil, CYC) for both disease forms. The treatment duration depends on disease form, clinical course, and disease activity.…”

Section: Discussionmentioning

confidence: 99%

Juvenile Scleroderma: A Referral Center Experience

Adroviç

2018

Arch Rheumatol

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Objectives: This study aims to evaluate the demographic and clinical features, laboratory data, treatment modalities, and outcomes of juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) patients at a referral pediatric rheumatology center in Turkey. Patients and methods: Medical records of a total of 57 patients, including 29 with JSS (1 male, 28 females; mean age 18.3±3.2 years; range 14 to 27 years) and 28 with JLS (6 males, 22 females; mean age 14.4±4.8 years; range 6 to 23 years), diagnosed betweenJanuary 2006 and Mart 2015 and followed-up for at least six months were evaluated in this retrospective longitudinal study. All medical records were retrospectively analyzed for demographic, clinical, and laboratory findings. Results: Mean age at disease onset was 9.9±4.2 years and 7.7±3.9 years for JSS and JLS, respectively. Mean ages at diagnosis and at the time of study were lower in JLS: 9.1±3.5 years vs. 11.7±3.7 years and 14.4±4.8 years vs. 18.3±3.2 years, respectively. Mean disease duration was 7.8±5.2 years and 8.0±4.3 years for JSS and JLS, respectively. Among JSS patients, interstitial lung disease was seen in eight (27%), pulmonary hypertension in three (10%), and arrhythmia in one (3%). One JSS patient (3%) died as a consequence of cardiac sclerosis. Corticosteroids with methotrexate were used in 29 JSS patients (100%) and in 21 JLS patients (75%). Patients with vasculopathy were treated with nifedipine (n=18, 62%) and bosentan (n=12, 41%). Internal organ involvement was treated with high-dose cyclophosphamide (n=10, 34%) or biological agent (n=3, 10%). Conclusion: Close monitoring of internal organ involvement is of great importance in preventing disease-related complications in JSS and JLS. Although rare, vital organ involvement has a devastating effect on prognosis. Biological agents represent an option for patients resistant to standard immunosuppressive treatment.

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Section: Discussionmentioning

confidence: 99%

Juvenile Scleroderma: A Referral Center Experience

Adroviç

2018

Arch Rheumatol

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“…Corticosteroids are not used in the treatment of gastrointestinal and vascular manifestations of systemic scleroderma, including Raynaud's phenomenon and digital ulcers. 4 Only a few articles describe the use of intralesional corticosteroids for limited cutaneous systemic scleroderma involving the orofacial region but, until now, there has been no literature on the use of a combination of intralesional corticosteroids and multiantioxidants for limited cutaneous systemic scleroderma.…”

Section: Introductionmentioning

confidence: 99%

A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face

Kumar¹,

Ms²,

Sh³

et al. 2016

BMJ Case Reports

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Limited cutaneous scleroderma is a subtype of scleroderma limited to the skin of the face, hands, feet and forearms. We present a case of a 45-year-old woman affected by limited cutaneous systemic scleroderma involving the orofacial region and causing restricted mouth opening. The patient showed noteworthy improvement of the skin lesion by use of a combination of intralesional corticosteroid with hyaluronidase and various multiantioxidants, resulting in amelioration of her mouth opening problem. The patient gave her full informed written consent to this report being published.

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“…Glucocorticoids (GC) are widely used in the general population [2] and represent a cornerstone of the treatment of many autoimmune systemic diseases [3][4][5]. Despite the lack of evidence of efficacy, GC are widely used in SSc patients, in particular in those with diffuse disease, interstitial lung disease (ILD), myositis, and arthritis [6][7][8][9], although a daily dosage below 15 mg of prednisone equivalent is often prescribed to minimize the risk of a scleroderma renal crisis [10]. As in other diseases, the use of GC is burdened by patient and physician concern about their long-term consequences [11][12][13], so that a tapering program can be planned when disease control is obtained [14].…”

Section: Introductionmentioning

confidence: 99%

Outcome of a glucocorticoid discontinuation regimen in patients with inactive systemic sclerosis

et al. 2016

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Glucocorticoids (GC) are widely used to treat systemic sclerosis (SSc). The lack of efficacy data and patient/physician concerns may prompt therapy discontinuation. The aim of this study is to identify factors hampering GC discontinuation in patients with stable disease on oral GC for longer than 12 months. Consecutive patients fulfilling the 2013 ACR/EULAR criteria for SSc and with stable disease were prescribed a slow tapering GC regimen to achieve discontinuation. At study entry and 6 months later (T6), patients were assessed for disease activity and severity. Moreover, the Short-Form-36; the Health Assessment Questionnaire Disability Index (HAQ-DI); and visual analog scales for fatigue, pain, and general health were completed. Reasons for stopping the discontinuation regimen were recorded. Forty-eight patients (46 females, 9 diffuse SSc), with a mean ± SD age of 56±14 years and a median disease duration of 10 years (range 2-22), were enrolled. The median daily GC dose was 5 mg (range 5-10; all patients treated with prednisone). At T6, 33 (68.7 %) patients had discontinued GC. The remaining 15 patients could not discontinue GC because of arthralgia in eight, arthritis in two, puffy fingers in two, increased creatine-kinase in two, and bursitis in one patient. At multiple logistic analysis, a higher baseline HAQ-DI was the only independent factor associated with GC need (OR 2.98, 95 % CI 1.20-7.41; p = 0.01). About one third of SSc patients did not achieve a GC-free regimen. Disability as assessed by HAQ-DI was the leading factor hindering GC discontinuation. A low HAQ-DI score can identify candidates for GC discontinuation.

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Corticosteroids in Myositis and Scleroderma

Cited by 13 publications

References 55 publications

Juvenile Scleroderma: A Referral Center Experience

Juvenile Scleroderma: A Referral Center Experience

A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face

Outcome of a glucocorticoid discontinuation regimen in patients with inactive systemic sclerosis

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