Recommendations of the Czech Society for Rheumatology for the diagnosis of systemic sclerosis


Authors: R. Bečvář 1;  T. Soukup 2;  J. Štork 3;  D. Suchý 4;  P. Němec 5;  P. Jansa 6;  Z. Fojtík 7;  P. Horák 8;  D. Němcová 9
Authors‘ workplace: Revmatologický ústav, Revmatologická klinika 1. LF UK, Praha 1;  II. interní gastroenterologická klinika, Fakultní nemocnice, LF UK, Hradec Králové 2;  Dermatovenerologická klinika, Všeobecná fakultní nemocnice, 1. LF UK, Praha 3;  Oddělení klinické farmakologie, Fakultní nemocnice, LF UK, Plzeň 4;  II. interní klinika Fakultní nemocnice u svaté Anny, LF MU, Brno 5;  II. interní klinika – klinika kardiologie a angiologie, Všeobecná fakultní nemocnice, 1. LF UK, Praha 6;  Interní hematologická a onkologická klinika Fakultní nemocnice, Brno 7;  III. interní klinika – nefrologická, revmatologická a endokrinologická, Fakultní nemocnice, LF UP, Olomouc 8;  Klinika dětí a dorostu, Všeobecná fakultní nemocnice, 1. LF UK, Praha 9
Published in: Čes. Revmatol., 22, 2014, No. 2, p. 51-68.
Category: Recommendation

Overview

Systemic sclerosis is a generalised connective tissue disease characterised by thickening and fibrosis of skin (scleroderma) and by typical multiorgan involvement. At the same time fibrotic changes occur in arterial vessel wall of all sizes. Capillaroscopy is an appropriate method to distinguish between primary and secondary Raynaud‘ phenomenon, while changes of macrovasculature can be proven using duplex or Doppler ultrasonography. Skin thickening can be evaluated by modified Rodnan skin score. The examination of locomotory systém is focused on joint contractures, range of joint movementi a detection tendon friction rubs. Radiographic examination can reveal thinning of distal falangi and acral osteolysis. The examination of gastrointestinal tract includes all methods used in gastroenterology. For esophagus additionally manometry and pH monitoring is performed. The examination of small intestine also comprises enteroclysis and abdominal CT. The involvement of lung parenchyma should be verified by pulmonary function tests (FVC, FEV1 and DLCO), further by chest radiography and HRCT. Bronchoscopy with lavage and cytology of the obtained fluid may contribute to diagnosis of alveolitis. Echocardiography is a key method on suspicion of pulmonary arterial hypertension. A positive finding should be verified by right heart catheterisation. Heart involvement can be proven using ECG and echocardiography, eventually perfusion scintigraphy is recommended. When renal manifestation is suspected blood pressure should be monitored and serum creatinine and urine analysis regularly performed. Permanent hypertension and proteinuria require examination of renal functions and renal biopsy. In juvenile form of SSc the spectrum of investigations is not different from that in adult form of the disease.

Key words:
Systemic sclerosis, Raynaud´s phenomenon, organ involvement, classification criteria, examination algorithms


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