Table 1

Clinical practice guidelines in systemic sclerosis: where do we stand?

Domain (n)Author and yearLevel of evidence10
Patients (0)No clinical practice guidelines were included.
Heart (3)Avouac et al, 201418 S2
Bissell et al, 201719 S2
Mavrogeni et al, 201620 S2
Malignancy (1) Lazzaroni et al, 201721 S2
Vascular and ulcers (6)Baron et al, 201422 S2
Fujimoto et al, 201623 S2
Hughes et al, 201524 S2
Pistorius et al, 201225 S1
Maverakis et al, 201426 S2
Cutolo et al, 201727 S1
Gastrointestinal (3)Alantar et al, 201128 S2
Baron et al, 201029 S1
Hansi et al, 201430 S2
Renal (1)Lynch et al, 201631 S1
Interstitial lung disease (0)No clinical practice guidelines were included.
Pulmonary arterial hypertension (2)Galiè et al, 201632 S3
Khanna et al, 201333 S3
Diagnosis (1)Knobler et al, 201734 S1
Contribution of healthcare providers (0)No clinical practice guidelines were included.
Treatment (5)Kowal-Bielecka et al, 201735 S3
Denton et al, 20166 S2
Sampaio-Barros et al, 201336 S2
Distler et al, 201137 S2
Knobler et al, 201438 S2
Haematopoietic stem cell transplantation (3)Rodrigues et al, 201339 S2
Saccardi et al, 200440 S2
Farge et al, 201741 S2
Clinical trials (2)Khanna et al, 201542 S3
Khanna et al, 201043 S1
Juvenile (1)Pain et al, 201644 S3
  • Level of evidence: Amendment of the ‘S-class’ classification is used to describe the degree of systematic development of guidelines and thus to identify their level of evidence, by the German Association of the Scientific Medical Societies Guidance Manual and Rules for Guideline Development:10 category S3: evidence-based and consensus-based guideline, based on a representative committee, systematic review andsynthesis of evidence (highest level of evidence); category S2: evidence-based or consensus-based guideline, based on either a systematic review and synthesis of evidence or a representative committee; category S1: recommendations by expert groups, based on consensus development in an informal procedure (lowest level of evidence).