Table 1.

Clinical signs, prevalence, and symptomatology in diffuse cutaneous systemic sclerosis (dcSSc).

Involved Organ(s)	Clinical Signs		Prevalence	Symptomatology
Vascular [4,5]	Raynaud’s phenomenon		100%	Changes in skin coloration
	Digital ulcers		41.6%	Hard-to-heal lesions → infection, osteomyelitis, gangrene
Cutaneous [6]	Dermal hardening		100%
	Pigmentation alterations			Patchy areas of depigmentation.
	Calcinosis cutis			Subcutaneous nodules Pain
Musculoskeletal [5]	Fibrosis		46–97%	Contractures Morning stiffness
	Arthritis			Arthralgias
Gastrointestinal [7]	Esophageal hypomotility		90%	Dysphagia
	Gastroesophageal reflux			Bacterial overgrowth
	Stomach hypomotility			Delayed emptying → Premature satiety, fullness, bloating, nausea
	Intestinal hypomotility			Chronic intestinal pseudo-obstruction Malabsorption
	Anorectal dysfunction			Incontinence
Renal [8]	Renal scleroderma crisis		10%	Malignant high blood pressure Angiopathic hemolytic anemia Thrombocytopenia Proteinuria Macrohematuria Acute renal failure
Cardiac [9]	Arrhythmias	Ventricular	90%	Fatigue Palpitations Syncope Dizziness
		Supraventricular	66%
		Ventricular multiform premature beats	40%
		Left bundle branch block	16%
		First-degree atrioventricular blocks	8%
	Pericardial conditions	Pericardial effusion	78%	Chest pain Dyspnea Fever
		Pericarditis	77.5%
	Myocardial dysfunction	RV dysfunction	69%	Fatigue Calf edema Dyspnea Venous congestion
		LV dysfunction	46%
		Cardiac failure	20–25%
	Valvular dysfunction	Mitral valves prolapse	20%	Dyspnea Pain Edema Palpitations Fatigue
Pulmonary [2]	Pulmonary interstitial disease		40%	Dyspnea Dry cough Crackles Pulmonary arterial hypertension Cor pulmonale
	Pulmonary arterial hypertension		15–20%	RV hypertrophy ↓ Cardiac output Heart failure

Abbreviations: RV: right ventricle; LV: left ventricle; ↓: decrease; →: produces.