Scleroderma overlap syndromes

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Current Opinion in Rheumatology 2002; 14(6):704-710

Scleroderma overlap syndromes

Janet E. Pope, MD, MPH, FRCPC

Scleroderma is a connective tissue disease that causes fibrosis and vascular

abnormalities, but that also has an autoimmune component. Many patients with

scleroderma have a positive antinuclear antibody, and there can be family

histories of other connective tissue diseases such as systemic lupus

erythematosus (SLE) and rheumatoid arthritis. Some patients have features of

scleroderma and other autoimmune conditions. This article will review recent

literature to help in the understanding of scleroderma with overlap

features. Recent reports of scleroderma overlap with rheumatoid arthritis

suggest distinct features of diffuse scleroderma with positive Scl-70,

pulmonary fibrosis, and later seropositive erosive rheumatoid arthritis. SLE

rarely occurs with scleroderma. Sjögren syndrome symptoms are common in

scleroderma. In primary Sjögren syndrome, anticentromere antibody positive

patients have more Raynaud phenomenon. Antibodies that occur in scleroderma

that are thought to be specific are present in other connective tissue

diseases. For instance, Scl-70 antibody is reported in as many as 35% of

patients with scleroderma but can be present in 25% of patients with SLE.

Myositis or myopathy can be features of scleroderma. Scleroderma overlap

with polymyositis is frequently anti-PM Scl antibody positive, whereas

anti-Jo-1 does not normally occur in the overlap of scleroderma and

polymyositis but is usually exclusively positive in polymyositis with

arthritis and alveolitis. A better prognosis is found with PM Scl antibody

in myositis. Vasculitis is not a typical feature of scleroderma, but has

been reported. Eosinophilic fasciitis is rare, and the onset could be

associated with simvastatin.

Abbreviations

ACA anticentromere antibody

aCL anticardiolipin antibody

ANA antinuclear antibody

anti-MPO anti-myeloperoxidase antibody

anti-SM anti-smith antibody

MCTD mixed connective tissue disease

MS multiple sclerosis

pANCA perinuclear antineutrophil cytoplasmic antibody

RA rheumatoid arthritis

RF rheumatoid factor

RNF ribonucleic protein

SLE systemic lupus erythematosus