Complications
Diminished forced vital capacity and low diffuse capacity for carbon monoxide precede ILD. Patients are often asymptomatic but may report dyspnoea, non-productive cough, and fatigue. Velcro-like crackles may be auscultated. High-resolution chest computed tomography usually shows non-specific interstitial pneumonia. Male sex, active smoking, and older age at presentation are associated with increased risk of disease progression and early mortality.[20] There is no definitive prevention strategy, but smoking should be discouraged and anti-reflux procedures can be instituted if aspiration is a risk. Influenza and pneumococcal vaccines are encouraged. ILD is treated with immunosuppressive agents (e.g., mycophenolate, cyclophosphamide, rituximab, tocilizumab, or nintedanib).[67][68][69] Rarely, corticosteroids or antifibrotic medications are used.[67]
Obliteration of the pulmonary arterioles with elevated pulmonary vascular resistance and right heart decompensation and death can occur at any time in the illness. Patients with older age at onset may be at increased risk. Echocardiograms should be done annually to screen for PAH and, if suspected, a right heart catheterisation done to make the diagnosis. PAH is treated with various classes of medications such as endothelin receptor antagonists, prostanoids, phosphodiesterase-5 inhibitors, and riociguat (soluble guanylate cyclase stimulator), and with heart-lung transplantation for some patients. Combination therapies are often used initially and a treat-to-target approach is considered to try to decrease the risk of progression to right heart failure.[70]
Severe cardiac involvement occurs in 10% to 30% of patients, and carries high mortality from associated congestive heart failure (CHF) or arrhythmia.[64][65][66] Fibrosis of the heart, pericardial effusion, or constrictive pericardial changes are typical findings. Treatment is dictated by clinical manifestations (e.g., CHF may be treated with pre- and after-load medications).
Fibrotic replacement and vasculopathy within the muscle or a myositis is typical. The prevalence of myopathy in systemic sclerosis is around 20%.[73][74][75] Proximal musculature is more involved than distal, and is difficult to treat, with an increased mortality including cardiomyopathy. Inflammatory myositis is extremely rare and often occurs in those with polymyositis/scleroderma auto-antibody. It does not usually respond to treatment.[76]
Small bowel bacterial overgrowth results from impaired peristalsis and the neutralisation of acid in the stomach from use of proton pump inhibitors. Symptoms include diarrhoea, bloating, gas, cramps, and foul-smelling stools, and sometimes constipation with intermittent diarrhoea. Treatment is antibiotics, generally given in a cyclic regimen in order to prevent drug resistance.[33]
A history of joint pain with swollen and tender joints suggests associated arthralgia and tenosynovitis, which is usually non-erosive but may be quite destructive with large erosions mimicking psoriatic arthritis. Treatment is anecdotal and ranges from non-steroidal anti-inflammatory drugs to corticosteroids, disease-modifying antirheumatic drugs, and occasionally tumour necrosis factor-alpha inhibitors and other biologicals (rituximab, tocilizumab, abatacept). Approximately 5% to 7% of patients with systemic sclerosis have rheumatoid arthritis.[71][72]
Carpal tunnel or median nerve entrapment is common where there is inflammatory arthritis or tenosynovitis. Typical median nerve entrapment numbness spares the fifth digit. Resting hand splints, injecting the carpal tunnel with corticosteroids, or surgery for median nerve release may be appropriate treatment options. There can be entrapment of other nerves such as trigeminal neuralgia, but this is extremely rare.
Sjogren's syndrome can occur at any time and is diagnosed by extent and severity of dry eyes, dry mouth, and parotid swelling. Salivary gland biopsy can differentiate between Sjogren's syndrome and fibrosis from systemic sclerosis. However, biopsy is not usually performed as it rarely changes treatment.
SRC is a medical emergency of new-onset hypertension with elevated creatinine and intravascular haemolysis. SRC affects 10% to 25% of patients with diffuse systemic sclerosis (SSc) and 1% to 2% of those with limited SSc.[63] Mortality from SRC has decreased from 80% to 20% and occurs acutely or within several months of diagnosis.[42][43] Survival is improved with the use of ACE inhibitors, which decrease renin and increase bradykinin (which vasodilates the renal vasculature), and hydration. See Management approach.
GAVE is a rare complication and often presents with iron-deficiency anaemia. It is managed with repeated treatments from the Yag laser.
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