The degree of skin involvement identifies distinct lung disease outcomes and survival in systemic sclerosis.

ABSTRACT:

Objective

To determine whether the pattern of skin involvement can predict clinical features, risk of restrictive lung disease (RLD) and survival in a large scleroderma (SSc) cohort.

Methods

Demographic and clinical data collected over 30 years from 2205 patients with SSc were retrospectively analysed after subdividing subjects into four subtypes based on pattern of skin fibrosis: type 0 (no skin involvement), type 1 (limited to metacarpophalangeal joints), type 2 (distal to elbows/knees) and type 3 (proximal to elbows/knees). Clinical features associated with skin subsets were identified by regression analyses. Kaplan-Meier and Cox proportional hazards models were used to compare time to RLD and survival across subtypes.

Results

The presence and severity of RLD were

SUBMITTER: Cottrell TR 

PROVIDER: S-EPMC4776325 | biostudies-literature | 2014 Jun

REPOSITORIES: biostudies-literature