Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

Figures & data

Figure 1 (A and B) demonstrate nailfold video-capillaroscopy findings in a patient with very early SSc who presented with RP and anticentromere antibodies. Arrows in (A) identify giant capillaries. (B) demonstrates capillary drop-out; the arrow in this panel identifies a microhemorrhage.

Table 1 Criteria for Very Early SSc Based on the LeRoy and Medsger Definition⁵ and the VEDOSS Criteria⁷

Features	LeRoy and Medsger Definition of Very Early SSc	VEDOSS Criteria for Very Early SSc
Raynaud Phenomenon	X	X
SSc-specific autoantibodies	X	X
SSc features on capillaroscopy	X	X
Puffy fingers		X
Positive ANA		X

Figure 2 The ACR/EULAR 2013 Classification Criteria for SSc, highlighting the criteria for very early SSc. A total score of 9 is sufficient to classify patients with definite SSc.

Table 2 Initial Evaluation of a Patient with Very Early or Early SSc

Category	Focused Evaluation Strategies
Patient history	Temporal evaluation of specific SSc symptoms (eg, Raynaud phenomenon, first non-Raynaud symptom, puffy fingers, tendon friction rubs, digital ulcers)
Physical examination	Thorough examination of all organ systems with special focus on skin and nailfold capillaries
Laboratory evaluation	Hemoglobin
	Creatinine
	Creatine kinase
	C-reactive protein
	Comprehensive SSc autoantibody panel
Diagnostic studies	Chest X-ray
	HRCT chest – highly recommended in all cases
	Transthoracic echocardiogram
	Pulmonary function tests
	Video-capillaroscopy
Invasive studies	Upper endoscopy in select cases

Figure 3 Flow chart for the evaluation of patients with early SSc by organ system with description of risk factors and diagnostic testing.

Table 3 Clinical Correlates of Autoantibody Profiles in SSc

Autoantibody	Cutaneous Subtype	SSc Features
Anti-Scl-70	Diffuse	Progressive ILD
Anti-RNA polymerase III	Diffuse	Renal crisis, GAVE
Anti-centromere	Limited	PAH
Anti-Th/To	Limited	ILD, PAH
U3-RNP	Limited and diffuse	PAH, inflammatory myositis
U11/U12 RNP	Limited and diffuse	ILD
Ku	Limited and diffuse	ILD, inflammatory myositis
Pm/Scl	Limited	Inflammatory arthritis, ILD, calcinosis, overlap syndromes (eg, myositis)
U1RNP	Limited	ILD, overlap syndromes (eg, mixed connective tissue disease)

Note: References for Table:^3,122–128.

Figure 4 Proteins related to very early SSc disease stages.

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