Out of 32 patients studied, there were 31 women, and one man. Age ranged from 22 to 67 years, with a mean of The demographic features of the three subgroups studied are in table 1. All patients had Raynaud's phenomenon and its onset ranged from zero to 13 years prior to diagnosis of SS. In the limited form, the mean time for developing Raynaud's before diagnosis was 2.
Involvement of hands was present in all patients as induration, except for one patient who had the edematous form. As to other cutaneous findings, their prevalence in the scleroderma population as a whole was: digital scars in 21 patients These data are presented in graph 1.
Recommendations for the management and treatment of systemic sclerosis
The results of these cutaneous findings according to the form of systemic sclerosis, in addition to frequency comparison in the different groups, are summarized in tables 2 and 3. The modified Rodnan skin score in the three forms represented a mean of SS is a relatively rare disease, but stands out for the variety of cutaneous findings that assist in making diagnosis.
In the population studied, patients with diffuse SS had a shorter period of disease than those with other forms, a fact that can be easily understood, given that in this form the higher aggressiveness of the disease makes patients seek doctors sooner. There was skin sclerosis in all patients and it was more severe in the diffuse form than in the limited one, which is translated as a higher modified Rodnan skin score.
As a matter of fact, this component relates to poorer prognosis regarding patient survival. Raynaud's phenomenon was present in all patients; this is a very important finding since it shows that its absence makes the diagnosis of SS less likely. The interval between the onset of Raynaud's phenomenon and diagnosis of SS was shorter in patients with the diffuse form, although the data did not show statistical significance. All other cutaneous manifestations studied, such as pigmentary changes, telangiectasia, microstomy, pruritus and calcinosis, were similarly observed in the different types of disease in the population studied, and the first two followed Raynaud's phenomenon and digital scars in prevalence.
Akenson et al. Tager et al. In conclusion, it could be emphasized that the most common cutaneous manifestations of SS in the present population, in addition to skin sclerosis itself, was Raynaud's phenomenon and digital scars. These findings should lead to inclusion of SS in the differential etiological diagnosis. Identifying associated cutaneous findings may help making presumptive diagnoses, even if they do not help distinguishing among the three subtypes. Wigley FM. Systemic Sclerosis: clinical features.
Primer on rheumatic diseases. Atlanta: Arthritis Foundation; Left ventricular diastolic function in systemic sclerosis. Calcium-channel blockers for Raynaud phenomenon in systemic sclerosis. Losartan therapy for Raynaud phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial. Sildenafil in the treatment of Raynaud phenomenon resistant to vasodilatory therapy. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist.
Prazosin for Raynaud phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev. Quigley EM, Quera R. Small intestinal bacterial overgrowth: roles of antibiotics, prebiotics, and probiotics. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma.
Impact of oral cyclophosphamide on health-related quality of life in patients with active scleroderma lung disease: results from the scleroderma lung study. Jain M, Varga J. Mycophenolate mofetil: a promising novel therapy in systemic sclerosis.
Curr Rheumatol Rep. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease. McLaughlin VV. Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest.
Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Efficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertension. J Cardiovasc Pharmacol. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. Long-term outcomes of scleroderma renal crisis. This content is owned by the AAFP.
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Sign Up Now. Previous: Vacuum-Assisted Vaginal Delivery. Oct 15, Issue. C 11 , 12 Doppler echocardiography, pulmonary function testing, and high-resolution computed tomography of the chest should be performed at diagnosis of systemic sclerosis and at regular intervals thereafter. C 7 , 8 Treating active interstitial lung disease with oral cyclophosphamide Cytoxan for one year modestly improves lung function, dyspnea, skin thickening, and health-related quality of life in patients with systemic sclerosis.
B 25 , 27 Initiation and continuation of angiotensin-converting enzyme inhibitors are recommended in patients with scleroderma renal crisis, even in the presence of elevated creatinine levels.
Towards a new classification of systemic sclerosis
Table 1 Distinguishing Clinical Features of Limited Cutaneous and Diffuse Cutaneous Systemic Sclerosis Feature Limited cutaneous Diffuse cutaneous Skin fibrosis Areas distal to the elbows and knees; may affect the face Areas proximal or distal to the elbows and knees; may affect the face Typical form of lung involvement Pulmonary arterial hypertension Interstitial lung disease Characteristic visceral organ Involvement Severe gastroesophageal reflux disease and Raynaud phenomenon Scleroderma renal crisis Physical examination Findings Telangiectasia, calcinosis cutis, sclerodactyly, digital ischemic complications Tendon friction rubs, pigment changes.
Table 2 Clinical Associations Between Systemic Sclerosis Subtypes and Scleroderma-Specific Autoantibodies Autoantibody Subtype percentage with subtype and autoantibody Clinical associations Antinuclear antibody Limited cutaneous and diffuse cutaneous 95 percent [nucleolar pattern is most specific] Pulmonary arterial hypertension Interstitial lung disease Anticentromere antibody Limited cutaneous 60 to 80 percent Pulmonary arterial hypertension Diffuse cutaneous 2 to 5 percent Digital ulcerations or digital loss Antitopoisomerase-1 antibody anti-Scl Diffuse cutaneous 20 to 40 percent Rapidly progressive skin thickening Scleroderma renal crisis Pulmonary fibrosis Information from references 3 and 4.
Table 4 System-Specific Involvement of Systemic Sclerosis System Manifestation History and physical examination findings Cardiovascular Abnormal cardiac conduction — Congestive heart failure Edema, extra heart sound S3 Diastolic dysfunction secondary to left ventricular fibrosis Pericardial effusion Digital ischemic changes Abnormal capillaries on the nail fold Acro-osteolysis Digital pitting or ulceration Pterygium inversus unguis i. Table 5 Characteristics of Primary and Secondary Raynaud Phenomenon Characteristic Primary Secondary Sex of patient Female Male or female Age of onset Adolescence Adulthood typically Symptom severity Mild to moderate Moderate to severe Physical examination findings Normal Abnormal capillaries on the nail fold capillaries best visualized using an otoscope ; digital pitting or ulcerations; pterygium inversus unguis i.
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Are you sure? More in Pubmed Citation Related Articles. Email Alerts Don't miss a single issue. Sign up for the free AFP email table of contents. Navigate this Article. Areas distal to the elbows and knees; may affect the face. Areas proximal or distal to the elbows and knees; may affect the face. Typical form of lung involvement. Pulmonary arterial hypertension. Characteristic visceral organ Involvement.
Severe gastroesophageal reflux disease and Raynaud phenomenon. Physical examination Findings. Tendon friction rubs, pigment changes. Anticentromere antibody. Limited cutaneous 60 to 80 percent. Digital ulcerations or digital loss. Antitopoisomerase-1 antibody anti-Scl Diffuse cutaneous 20 to 40 percent.
Rapidly progressive skin thickening. Diffuse cutaneous systemic sclerosis. Limited cutaneous systemic sclerosis. Systemic sclerosis sine scleroderma. Localized scleroderma.
Epidemiology and Classification
Mixed connective tissue disease. Features of systemic sclerosis, polymyositis, and SLE. Systemic sclerosis plus polymyositis, rheumatoid arthritis, or SLE. Undifferentiated connective tissue disease. Abnormal cardiac conduction. Congestive heart failure. Edema, extra heart sound S3. Diastolic dysfunction secondary to left ventricular fibrosis.
Digital ischemic changes. Abnormal capillaries on the nail fold. Digital pitting or ulceration. Barrett esophagus or strictures. Gastric antral vascular ectasia watermelon stomach. Gastrointestinal bleeding. Gastroesophageal reflux disease. Intestinal malabsorption. Dyspareunia, impotence. Long-standing Raynaud phenomenon is a lesser risk factor for the development of scleroderma.
Latest Research and Reviews
The earliest description of scleroderma skleros , hard; derma , skin may have been made by Hippocrates around BCE Aphorism V He noted, "In those persons in whom the skin is stretched, parched, and hard , the disease terminates without sweats. In Raynaud commented on the occurrence of peripheral vasospastic events in an individual with scleroderma.
Reports of occasional visceral lung, kidney, gastrointestinal, and cardiac involvement in some cases of scleroderma were made in the late s and early s. In Thomas noted the association of calcinosis, Raynaud phenomenon, esophageal dysfunction, and telangiectasias in an individual with scleroderma. CREST is an acronym for calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias. Recognized subsets of scleroderma include: diffuse systemic sclerosis, limited cutaneous systemic sclerosis, limited, localized morphea and linear scleroderma , overlap syndromes, and undefined connective tissue disease Wigley ; Mayes The classification system is based on a scoring system with different weight given to various clinical features and the presence of antibodies associated with scleroderma.
Important exclusion criteria include patients who have scleroderma-like disorders or patients with skin thickening that spares the fingers. Skin thickening of the fingers of both hands extending proximally to the metacarpal phalangeal joints carries the most weight in the classification criteria.