5th June 2025

Complications

The greatest complications of systemic sclerosis are interstitial lung disease (ILD) and renal crisis. Development of ILD will need specialist referral and can ultimately require lung transplantation. 

Severe and life-threatening renal disease occurs in 10-15% of patients and was the largest mortality cause before ACE inhibitors (ACEi) were introduced. Early dcSSc patients are at highest risk of developing a renal crisis. The signs of a renal crisis are onset of acute renal failure, abrupt onset of moderate or marked hypertension, urinalysis indicating proteinuria with few cells or casts and micro-angiopathic haemolytic anaemia. ACEi must be titrated to the maximal tolerated dose and if required, further anti-hypertensives can be added (usually calcium channel blockers). 

Small pericardial effusions can occur when cardiac involvement is noted, which can progress to cardiac tamponade. Oral prednisolone is given for pericardial effusions, but strict monitoring is required, as corticosteroids can precipitate a renal crisis. Tamponade is managed with a pericardial window and systemic corticosteroids.

Patients should be monitored with regular ECHOs to look for the development of pulmonary hypertension, with referral for right heart catheterisation if needed.

Raynaud’s phenomenon can lead to digital ulceration and skin sclerosis can cause fixed contractures. Patients may develop gastric antral vascular ectasia (GAVE). Heartburn can lead to strictures and Barrett’s metaplasia and needs early intervention.