Systemic Sclerosis Sine Scleroderma PDF Print E-mail
Saturday, 24 April 2010 21:24
HADI POORMOGHIM, MARY LUCAS, NOREEN FERTIG, and THOMAS A. MEDSGER, JR.
ARTHRITIS & RHEUMATISM Vol. 43, No. 2, February 2000, pp 444–451 © 2000, American College of Rheumatology

Objective
To describe the demographic, clinical, and laboratory features and natural history of patients with systemic sclerosis sine scleroderma (ssSSc), and to compare them with those of patients with SSc and limited cutaneous involvement (lcSSc).

Methods
The University of Pittsburgh Scleroderma Databank served as the data source. Patients were divided into those who had no skin thickening (ssSSc) and those who had skin thickening only distal to elbows or knees and/or of the face (lcSSc) during their disease course. These two groups were compared with regard to demographic characteristics, clinical, laboratory, and serologic features, and survival rates. Chisquare and Student’s t-test analyses were performed, and Fisher’s exact test was used as appropriate.

Results
Of 555 consecutive patients without diffuse cutaneous SSc, 48 (9%) had ssSSc and 507 (91%) had lcSSc. The ssSSc patients had a mean total disease duration of 18.6 years (15.1 years before study entry and 3.5 years of followup after study entry), and had not developed lcSSc or another connective tissue disease. Other than the absence of skin thickening, the ssSSc group had no significant differences in individual internal organ involvements, laboratory features, serum autoantibody type, or survival rate compared with patients with lcSSc.

Within the category of lung involvement, patients with ssSSc had a significantly greater frequency of dyspnea with mild exertion or at rest, and a tendency toward reduced carbon monoxide diffusing capacity (<70% of predicted normal) and primary pulmonary arterial hypertension. Patients with lcSSc had significantly more frequent individual manifestations of digital pitting scars, digital-tip ulcers, telangiectasia, and calcinosis than those with ssSSc, in part related to increased time of observation. Puffy fingers and finger joint contractures were detected significantly more often in lcSSc patients.

Conclusion
Systemic sclerosis sine scleroderma should be included in the spectrum of SSc with limited cutaneous involvement and should not be considered a distinct or separate disorder.

Login to download your own full copy of the article from the link provided below.

 
More articles :

» Development of an Activity Index for Localized Scleroderma (Morphea)

E. Lanciano, A. CHIALà, E. Praino, V. Grattagliano, M. Covelli, F. Iannone, G. Lapadula Reumatologia Universitaria, Policlinico, Bari, ItalyAnn Rheum Dis 2011;70(Suppl3):481Background: Morphea is a skin disorder characterized by fibrosis. It...

» Cancer Drug Shows Promise For Treating Scleroderma

A drug approved to treat certain types of cancer has shown promising results in the treatment of patients with , according to results from an open-label Phase II trial. While the drug's efficacy must be demonstrated in a Phase III trial, the gold...

» Greater Global Cooperation and Collaboration in Scleroderma Research Required

Greater global cooperation and collaboration among Scleroderma researchers and clinicians would be all that is required to fuel a drive toward important discoveries and optimize outcomes for patients with this chronic, progressive, and often...

» Our Donors

The Scleroderma Care Foundation wishes to express our appreciation and gratitude for the generosity and support extended to us, by all of our donors to date, and the list keeps on growing. Your contributions have all been incredibly helpful and...

» Subclinical Atherosclerosis Noted in Diffuse Scleroderma

Patients with diffuse systemic sclerosis (SSc), without any clinical evidence of cardiovascular disease, have indicators of subclinical , according to a study published online Aug. 16 in Arthritis Care & Research.To identify signs of early...

» Scleroderma Foundation's 13th Annual National Patient Education Conference Is Here

Nearly 600 people are anticipated to visit the heart of San Francisco Friday, July 8, through Sunday, July 10, for the Scleroderma Foundation's 13th Annual National Patient Education Conference. Patients, family members, caregivers, doctors and...