Question

Write assessment and current evidence-based treatment modalities for scleroderma-ILD.

Hint:

Early diagnosis and treatment with immunosuppressive therapies are essential for managing scleroderma-ILD and preventing irreversible lung damage.

Answer 1

Step 1: Assessment of Scleroderma-ILD.
The assessment of scleroderma-ILD includes a combination of clinical, radiological, and pulmonary function tests. Key steps include:
Step 2: Diagnostic Approaches.
- Clinical evaluation: Detailed history and physical examination to assess symptoms of ILD, such as dyspnea, dry cough, and fatigue.
- Pulmonary function tests (PFTs): Measurement of lung volumes, FVC, and DLCO to assess the degree of restriction and gas exchange impairment.
- High-resolution computed tomography (HRCT): HRCT is the gold standard for evaluating interstitial lung changes, detecting early fibrosis, and monitoring disease progression. - Serological tests: Anti-Scl-70 antibodies, along with other markers, can help assess the risk of developing ILD.
Step 3: Treatment Modalities.
The treatment of scleroderma-ILD focuses on controlling inflammation, slowing down fibrosis, and improving symptoms:
- Immunosuppressive therapy: Methotrexate, cyclophosphamide, and mycophenolate mofetil (MMF) are commonly used to treat inflammation and reduce fibrosis.
- Monoclonal antibodies: Rituximab and tocilizumab have shown promising results in reducing disease progression in some cases of scleroderma-ILD.
- Lung transplantation: In advanced cases of scleroderma-ILD with progressive pulmonary fibrosis, lung transplantation may be considered.
Step 4: Current Evidence.
Recent evidence supports the use of mycophenolate mofetil (MMF) for long-term treatment, particularly in preventing disease progression. Clinical trials are ongoing to evaluate the efficacy of newer biologic therapies and combination treatments.