26th January 2024

Management 

The management of systemic sclerosis depends on the disease subset and the presence or absence of internal organ involvement.

Patients with diffuse skin involvement or inflammatory organ involvement are treated with systemic immunosuppressive therapy. These patients may have skin involvement that is severe or diffuse, interstitial lung disease, myocarditis, or arthritis.

Methotrexate and mycophenolate mofetil are both used in patients who exhibit diffuse and progressive skin involvement but no visceral involvement. Generally, methotrexate is used in patients with arthritis or myositis, while mycophenolate mofetil is used in those who have significant interstitial lung disease. For patients who are refractory to the use of these, cyclophosphamide is usually the next step.

In cases of renal sclerosis, the mainstay of treatment is angiotensin-converting enzyme inhibitors (ACE-i). Initially, Captopril may be stated acutely for example, in renal scleroderma crisis within the first 72 hours, after which ACE-i such as Enalapril or Ramipril are used for maintenance. It is essential to closely monitor the serum creatinine (small, transient increases are expected and not usually an indication to stop therapy). There is no evidence that supports the use of ARBs or non-ACE-i therapy.

Gastrointestinal manifestations are treated symptomatically. For instance, proton pump inhibitors are used for oesophageal reflux and gastritis and can be titrated to up to twice daily. Oesophageal dysmotility, which may present with dysphagia, can be treated using prokinetic drugs such as Domperidone and Metoclopramide. Small intestinal bacterial overgrowth is another sequela and is usually treated with antibiotics, namely Metronidazole.

Raynaud’s phenomenon, either because of systemic sclerosis or primary RP, is treated with long-acting dihydropyridine calcium channel blockers such as Amlodipine or Nifedipine. If calcium channel blockers are not tolerated or ineffective, phosphodiesterase type 5 inhibitors such as Sildenafil may be used. Topical nitrates can be considered for patients with ischemic digits as a result of RP. However, it is not recommended to use topical nitrates with PDE 5 inhibitors due to the risk of hypotension.

Arthralgia and arthritis are treated initially with NSAIDs or Paracetamol for symptomatic relief. However, if these are contraindicated or ineffective, alternatives such as low-dose prednisone or hydroxychloroquine can be used. Methotrexate is another option, but only in those patients who are not at risk of pulmonary fibrosis.