Acral changes include RP, sclerodactyly, soft-tissue thinning, digital pits and ulcers, and acroosteolysis . Angio-MRI is being developed to study large-caliber distal vessels in the hands. (Figure 1)
RP is observed in more than 95% of ScS , most frequently affects the fingers of the hand (and may be complicated by trophic disorders such as finger ulcers or pulpy depressed scars.
Telangiectasias are dilations of the small skin vessels, rounded, located in the face, lips, tongue or palm of the hands.
Peri-ungual capillaroscopy could be done during the association of inflammatory polyarticular damage and a Raynaud phenomenon to diagnosis a scleroderma pattern in the classification of capillary abnormalities (major capillary dystrophies, megacapillaries, capillary rarefaction). Angio-MRI is interesting to study large-caliber distal vessels in the hands, allowing an analysis of arterial, venous and tissue time (6). Thermography, doppler and laser, could help assess microcirculatory vascular involvement and small vessels in the hands , however this methods are being studied
The first line cardiac exam is doppler ultrasound; yet Cardiac MRI is potentially very useful in cardiac assessment if primary myocardial damage is suspected (Figure.2,3) .Chest CT is the exam that allows evaluation of lung involvement
In fact Pulmonary arterial hypertension affects 7-8% of patients (3), and heart failure affects 5.4% (4).
Natriuretic factors are the first-line biologic markers for detecting cardiac distress (7).
The second exam is Doppler Ultrasound, which allows the diagnosis of systolic and diastolic left ventricular dysfunction, by analysing ventricular ejection fraction , filling pressures , pulmonary arterial pressure estimation, right ventricle (Figure 3) and the pericardium.
However if pulmonary arterial hypertension (PAH) is suspected right catheterization become the recommended examination , it directly measures pulmonary arterial pressure and determines the mechanism by measuring capillary pressures.
If primary myocardial involvement is suspected, nuclear MRI can identify micro-circulatory ischemic damage (Figure 3A), possible myocarditis (Figure 3B) or fibrous lesions (Figure 3C).
The high resolution millimetre slice chest CT is the reference exam performed to look for lung damage.
The main radiological elements of ILD are: pulmonary opacities in frosted glass , reticular opacities, subpleural ,cystic images, interstitial or linear micronodular opacities and traction bronchiectasis (Figure 4)
Musculoskeletal system disorders also requires imaging, Calcinosis can be evaluated by computed tomography.(Figure 5)
Joint disease is the first sign of the disease after the Raynaud phenomenon.
The joint involvement is poly-articular, touching in the first place the hands the wrists, the knees : bone erosions or joint pinches that first touch the hands. Lesions often predominates in metacarpal-phalangial and distal interphalangeal areas. (Figure 6) (8).
Tendon impairment is a criterion of severity , can be seen in the diffuse form of ScS. it is a fibrous disease of tendon sheaths and aponeuroses. it is associated with severe visceral damage (9).
Calcinosis is characterized by deposits of hydroxyapatite crystals in soft tissues. It is observed in 20-30% of patients, both in the limited and diffuse form of ScS (10).
Ultrasound allows detection of synovites and tenosynovites of the ScS (11). Fibrous tenosynovitis has a particular aspect in ultrasound characterized by a peritendinous thickening “in the heart of artichoke” with concentric lines around the tendinous body (12) (Figure 7). Assessment of the hands can be difficult due to skin sclerosis . MRI is then an interesting alternative, allowing to detect synovites, tenosynovites, but also bone erosions and bone edema (13).