From the Guidelines
The recommended systemic assessment and treatment approach for patients with limited scleroderma involves a comprehensive multidisciplinary care plan tailored to specific organ involvement, as outlined in the 2023 EULAR recommendations update 1.
Systemic Assessment
The initial evaluation should include:
- Complete blood count
- Comprehensive metabolic panel
- Urinalysis
- Pulmonary function tests with DLCO
- High-resolution chest CT
- Echocardiogram
- Nailfold capillaroscopy
Organ-Specific Treatment Approach
For Raynaud's phenomenon, first-line treatment includes dihydropyridine-type calcium antagonists, such as nifedipine, and PDE5 inhibitors should also be considered 1.
- Gastrointestinal manifestations require proton pump inhibitors (PPI) like omeprazole for reflux and prokinetic agents such as metoclopramide for dysmotility.
- Pulmonary arterial hypertension may require a combination of PDE5 inhibitors and endothelin receptor antagonists as first-line treatment 1.
- For interstitial lung disease, mycophenolate mofetil (MMF) or cyclophosphamide should be considered, with nintedanib also being an option 1.
- Digital ulcers can be managed with vasodilators, such as PDE5 inhibitors and/or intravenous iloprost, and wound care.
- Skin manifestations may benefit from methotrexate, mycophenolate mofetil, and/or rituximab 1.
Monitoring and Follow-Up
Regular follow-up every 3-6 months is essential to monitor disease progression and treatment response, with annual screening for pulmonary hypertension.
- This approach targets the vasculopathy, fibrosis, and immune dysregulation that characterize limited scleroderma, addressing both symptom management and disease progression.
From the Research
Systemic Assessment for Limited Scleroderma
The recommended systemic assessment for patients with limited scleroderma involves a comprehensive evaluation of the disease's clinical manifestations and potential organ involvement.
- Limited scleroderma, also known as CREST syndrome, is characterized by skin thickening limited to the hands, digital ulcers, and esophageal dysmotility 2.
- Patients with limited scleroderma may also experience life-threatening complications, such as small intestine hypomotility and pulmonary hypertension 2.
- Systemic sclerosis, including limited scleroderma, can affect various internal organs, including the lungs, gastrointestinal system, kidneys, and heart 3.
- Autoantibodies can be used to predict phenotype and internal organ involvement in systemic sclerosis 3.
Treatment Approach
The treatment approach for limited scleroderma focuses on improving quality of life by minimizing specific organ-threatening involvement and life-threatening disease.
- Early management of systemic sclerosis is crucial to prevent progression 3.
- Numerous therapeutic interventions exist, but there is no cure for systemic sclerosis 3.
- Treatment options may include glucocorticoids, immunosuppressants, and vasodilators to target vascular damage, fibrosis, and regulation of inflammation 4.
- Biologic agents targeting collagen, cytokines, and cell surface molecules may also have promising therapeutic effects in systemic sclerosis 4.
- Hematopoietic stem cell transplantation (HSCT) has shown promise as a treatment option for severe and refractory systemic sclerosis 4.
- Local skin-targeted therapy, including topical application of glucocorticoids or other immunomodulatory ointments and ultraviolet (UV) irradiation, may be used for localized scleroderma 4.
- Systemic immunosuppressants may also be utilized in several forms of localized scleroderma 4.
- Improved understanding of systemic sclerosis has allowed for better management of the disease, including improved classification and more systematic assessment and follow-up 5.
- Treatments for specific complications, such as scleroderma renal crisis, pulmonary arterial hypertension, digital ulceration, and gastro-oesophageal reflux, have emerged 5.