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 :

» Biomarkers Predict Lung Decline in Systemic Sclerosis

Predicting risk of pulmonary fibrosis and pulmonary hypertension is an urgent priority in systemic sclerosis. Lung function often declines rapidly in the first years after diagnosis, and interstitial lung disease (ILD), although highly variable in...

» Making A Difference

, a Center for Student Missions team member, recently recounted a touching experience, in which she heard about and met someone with Scleroderma for the very first time. According to Jessica, "We don't always get to see the effect of what it is we...

» Long-Term Outcomes of Scleroderma Renal Crisis

In a previous study. researchers sought to describe the medical history of a group of patients with Scleroderma Renal Crisis who had been treated with ACE inhibitors. The following is essentially a summary of that study, which was conducted by...

» The Role of Intravenous Immunoglobulin Preparations in the Treatment of Systemic Sclerosis

Marta Baleva and Krasimir NikolovReceived 12 June 2011; Revised 28 August 2011; Accepted 28 August 2011Scleroderma is progressive autoimmune disease associated with severe disability. The major underlying pathological process in Scleroderma is...

» Is Pulmonary Arterial Hypertension Really a Late Complication of Systemic Sclerosis?

Eric Hachulla, MD, PhD; David Launay, MD, PhD; Luc Mouthon, MD, PhD; Olivier Sitbon, MD, PhD; Alice Berezne, MD; Loïc Guillevin, MD; Pierre-Yves Hatron, MD; Ge´rald Simonneau, MD; Pierre Clerson, MD; and Marc Humbert, MD, PhD;Pulmonary arterial...

» January 26th Lunch & Learn Event on Sjogren’s Syndrome

For those of you in the state of Texas, Northwest Rheumatology, a division of Northwest Diagnostic Clinic, will host a free Lunch & Learn event on Sjogren’s Sydrome: The Forgotten Disease on Tuesday, Jan. 26, from 11:45 a.m. – 1 p.m. at the...