26th January 2024

Presentation

Cutaneous Manifestations

Skin involvement is a universal feature which commonly presents early in the face and hands. Features include:

• Tight/taut skin 
• Shiny skin
• Pigmentation changes – hyperpigmentation and depigmentation 
• Telangiectasia
• Lipoatrophy 

Image Source: Wikimedia Commons (2022). Systemic sclerosis 2  fingers, Licensed under a creative commons licence. Available for free at: https://upload.wikimedia.org/wikipedia/commons/1/15/Systemic_sclerosis%2C_fingers.jpg (Accessed: 10 July 2022).

Musculoskeletal manifestations 

• Swelling and stiffness of the fingers 
• Large and small joint contractures
• Joint pain and immobility 
• Arthritis 
• Tendonitis 
• Tendon friction rubs 
• Calcinosis cutis (soft tissue calcifications and resorption of the distal phalangeal tufts can be seen on radiographs) 

Image Source: Wikimedia Commons (2022). Calcinosis of CREST syndrome, Licensed under a creative commons licence. Available for free at: https://upload.wikimedia.org/wikipedia/commons/c/c8/Calcinosis_of_CREST_syndrome.jpg (Accessed:10 July  2022).

Vascular manifestations

Digital ischemic ulcers can develop in up to 50% of patients with systemic sclerosis. Progressive changes within the small blood vessels occur and can cause blood flow to be impaired permanently. It is more commonly seen in patients with diffuse disease and those with positive anti-topoisomerase I (Scl-70) antibodies. This can result in gangrene, infection and, in extreme cases, amputation. 

Gastrointestinal manifestations:

Almost 90% of patients with systemic sclerosis have evidence of gastrointestinal involvement, and the oesophagus is the most frequently affected area. Symptoms include dysphagia, early satiety, bloating, constipation, small bowel bacterial overgrowth, pseudo-obstruction, faecal incontinence and gastro-oesophageal reflux. 

Gastrointestinal bleeding and subsequent anaemia may occur due to angiodysplasia in the antrum of the stomach, which is sometimes referred to as “watermelon stomach.” 

Pulmonary manifestations

Pulmonary involvement may be seen in more than 80% of patients and mainly manifests as interstitial lung disease or pulmonary fibrosis. This can eventually lead to pulmonary arterial hypertension.

Renal manifestations

Renal involvement may occur in 50-80% of patients and can present with microalbuminuria, mild elevation in plasma creatinine or hypertension. Rarely (in 10-15% of patients) scleroderma renal crisis can occur and is characterised by malignant hypertension, acute oliguria, microangiopathic haemolysis, thrombocytopenia and mild proteinuria.