Juvenile systemic sclerosis severity score

	0 (normal)	1 (mild)	2 (moderate)	3 (severe)	4 (end-stage)	Maximum possible score
General*	BMI ≥ baseline Hgb >11.5 g/dL	BMI <1^st centile Hgb 10 to 11.4 g/dL	BMI <2^nd centile Hgb 9 to 9.9 g/dL	BMI <3^rd centile Hgb 7 to 8.9 g/dL	BMI <4^th centile Hgb <7 g/dL	4
Vascular	No Raynaud	Raynaud requiring vasodilators	Digital tip scars	Digital tip ulcerations	Digital gangrene	4
Skin	MRSS 0	MRSS 1 to 14	MRSS 15 to 29	MRSS 30 to 39	MRSS >40	4
Osteoarticular^¶	No articular involvement		Presence of limited range of motion		Presence of arthritis and/or tendon friction rub	2
Muscle^¶	Normal proximal muscle strength	CMAS 39 to 51	CMAS 26 to 38	CMAS 13 to 25	CMAS 0 to 12	2
GI*	Normal results of proximal GI tract investigations	GI symptoms Distal esophageal hypomotility GERD on 24-hour Ph-metry or scintiscan	Medium and/or high esophageal hypomotility	Malabsorption syndrome	Hyperalimentation	4
Respiratory^Δ	DLco >80% FVC >80% Normal HRCT sPAP <30 mmHg	DLco 70 to 79% FVC 70 to 79% Ground glass changes on HRCT sPAP 31 to 45 mmHg	DLco 50 to 69% FVC 50 to 69% Honeycomb changes on HRCT sPAP 46 to 75 mmHg	DLco <50% FVC <50% Fibrosis on radiography sPAP >75 mmHg	O₂ dependence	8
Cardiac^Δ	Normal ECG LVEF >50%	ECG conduction defect LVEF 45 to 49%	Arrhythmia LVEF 40 to 44%	Arrhythmia requiring treatment LVEF 30 to 39%	Congestive heart failure LVEF <30%	8
Renal^◊	GFR >90 mL/minute	GFR 75 to 89 mL/minute	GFR 50 to 74 mL/minute	GFR 10 to 49 mL/minute	End-stage renal failure	4

BMI: body mass index; HgB: hemoglobin; MRSS: modified Rodnan skin thickness score; CMAS: Childhood Myositis Assessment Scale; GI: gastrointestinal; GERD: gastroesophageal reflux disease; DLco: diffusing capacity for carbon monoxide; FVC: forced vital capacity; HRCT: high-resolution computed tomography; sPAP: estimated systolic pulmonary artery pressure by Doppler echocardiography; ECG: electrocardiogram; LVEF: left ventricular ejection fraction; GFR: glomerular filtration rate.
* Any of the parameters defines the score.
¶ Each score should be multiplied by 0.5 to obtain the final score.
Δ Any of the parameters defines the score. Each score should be multiplied by 2 to obtain the final score.
◊ The GFR was used to measure creatinine clearance. Values are calculated as follows: for males ages 0 to 12 years, 0.55 x height (cm)/creatinine (mg/dL); for males ages 12 to 18 years, 0.7 x height (cm)/creatinine (mg/dL); for females age 0 to 18 years, 0.55 x height (cm)/creatinine (mg/dL); for males older than age 18 years, (140 - age) x weight (kg)/(72 x creatinine [mg/dL]); for females older than age 18 years, (140 - age) x weight (kg)/(72 x creatinine [mg/dL]) x 0.85.

From: La Torre F, Martini G, Russo R, et al. A preliminary disease severity score for juvenile systemic sclerosis. Arthritis Rheum 2012; 64:4143. http://onlinelibrary.wiley.com/doi/10.1002/art.34652/abstract. Copyright © 2012 American College of Rheumatology. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (http://onlinelibrary.wiley.com).

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Juvenile systemic sclerosis severity score