Screening
There is no screening to determine if someone has systemic sclerosis (SSc); however, patients with severe/complicated Raynaud phenomenon (RP) should be screened for secondary causes by history and physical exam. The presence of dilated capillaries should be determined at the nailbed, often with magnification, and an antinuclear antibody test may be ordered. Patients who are anticentromere antibody positive and have RP have a 30% chance of developing SSc (often the limited cutaneous SSc subset) over the next 5 years, and this increases if they have dilated capillaries at the nail folds.[27]
Screening for complications in patients with established SSc
Annual echocardiograms are recommended to screen for pulmonary arterial hypertension (PAH) in SSc. Brain natriuretic peptide (BNP) can be used as a screen for PAH in SSc and to follow disease progression. However, it is neither specific nor diagnostic and is increased, for example, in cardiomyopathy. Nevertheless, worsening BNP is related to worsening PAH in SSc that is associated with PAH.[28]
Some experts also recommend annual pulmonary function tests, as a very low diffusing capacity (DLCO) can increase the likelihood of PAH. If both DLCO and forced vital capacity are equally reduced, the possibility of interstitial lung disease (ILD) is increased. See Idiopathic pulmonary arterial hypertension.
High-resolution computed tomography scan of the chest may be performed to screen for ILD at diagnosis and if there are new symptoms that might indicate ILD, or if there is a clinically significant decline in pulmonary function.[19][20][21] Antitopoisomerase I antibodies have been associated with an increased risk of severe ILD.[22]
Blood pressure (BP) monitoring, including at home, is especially important if skin involvement is worsening or if corticosteroids are being used (both are risk factors for scleroderma renal crisis). A target BP of <135/85 mmHg is desirable. Complete blood count should be done every 6 to 12 months to rule out iron-deficiency anemia. Routine endoscopies are recommended if Barrett esophagus and increased risk of cancer exist.
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