The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases

doi:10.1016/S0002-9343(96)00335-X

The American Journal of Medicine

Volume 101, Issue 6, December 1996, Pages 576-583

https://doi.org/10.1016/S0002-9343(96)00335-X Get rights and content

Purpose

To determine the prevalence and clinical associations of anticardiolipin antibodies (aCL) in a blinded, controlled study of patients with a variety of connective tissue diseases (CTD) using a standardized aCL testing system.

Patients and methods

Anticardiolipin antibodies (IgG, IgM, and IgA) were measured by direct enzyme-linked immunosorbent assay (ELISA) in the baseline serum samples of patients enrolled in a Cooperative Study of Systematic Rheumatic Diseases (CSSRD), National Institutes of Health (NIH) supported, 5-year inception-cohort, prospective study of early rheumatic diseases: rheumatoid arthritis (RA, n = 70), systemic lupus erythematosus (SLE, n = 70), scleroderma (PSS, n = 45), myositis (PM/DM, n = 36), and early undifferentiated connective tissue disease (EUCTD, n == 165). Diagnosis was based on standardized criteria and determined at the last study visit. A nested group of patients with Sjögren's syndrome (SJ, n = 44) was also defined. Serum from 200 blood donors (BB) served as controls. Additional patients with known antiphospholipid syndrome (APS, n = 33) and ANCA-related renal vasculitis (ANCA, n = 52) were also studied. Laboratory personnel were blinded to sample diagnostic group.

Results

The prevalence of either IgG or IgM aCL among each diagnostic group was RA 15.7%, SLE 15.76%, PSS 6.7%, PM/DM 8.3%, EUCTD 9.1%, SJ 6.8%, ANCA 3.8%, and BB controls 4.0%. Prevalence of aCL was significantly different for both the RA and SLE groups versus BB controls (P <0.01) but not among other diagnostic groups. Only 2 study patients had positive tests for IgA aCL (1 with PM/DM and 1 with EUCTD) versus 15% of APS with positive IgA aCL. Study patients positive for IgG or IgM aCL were significantly more likely to have hemolytic anemia or a positive serologic test for syphilis and less likely to have Raynaud's phenomenon. However, no associations were found between aCL positivity and thrombocytopenia, seizures, renal insufficiency, presence of a positive antinuclear antibody or rheumatoid factor, subcutaneous nodules or digital ulcers.

Conclusions

Based on results from this large CSSRD inception cohort, anticardiolipin antibodies are present in approximately 16% of patients with RA or SLE but are less common in patients with PSS, PM/DM, EUCTD, SJ, and ANCA vasculitis, where their prevalence approaches that in the normal population. Few consistent clinical associations can be found among patients with CTD who are aCL positive. The complete diagnostic and prognostic importance and specificity of these antibodies remains to be fully determined.

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^**: Dr. Merkel was supported by a Clinical Associate Physician Award from the General Clinical Research Centers Program of the National Center for Research Resources of the National Institutes of Health; Grant No. M01 RR01066.

View full text

Clinical studyThe prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases**

Purpose

Patients and methods

Results

Conclusions

Rheum Dis Clin North Am

Rheum Dis Clin North Am

Clin Immunol Immunopathol

Lancet

J Am Acad Derm

J Am Acad Derm

The “primary” antiphospholipid syndrome: major clinical and serological features

Medicine

Early undiffereritiated connective tissue disease. I. Early clinical manifestations in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease

J Rheumatol

Early undiffereritiated connective tissue disease. II. The frequency of circulating antinuclear antibodies in patients with early rheumatic diseases

J Rheumatol

Early undifferentiated connective tissue disease. III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis)

Ann Intern Med

Preliminary criteria for the classification of systemic lupus erythematosus

Bull Rheum Dis

1958 revision of diagnostic criteria for the classification of rheumatoid arthritis

Bull Rheum Dis

Polymyositis and dermatomyositis (first of two parts)

NEJM

Preliminary criteria for the classification of systemic sclerosis (scleroderma)

Arthritis Rheum

Definition of elements pertaining to general history

Special report. The second international anti-cardiolipin standardization workshop/the Kingston anti-phospholipid antibody study (KAPS) group

Am J Clin Path

Anti-phospholipid antibodies in syphilis and a thrombotic subset of SLE: distinct profiles of epitope specificity

Clin Exp Immunol

Anticardiolipin antibodies in patients with rheumatoid arthritis

Clin Rheumatol

Anticardiolipin and antineutrophil antibodies in giant cell arteritis

J Rheumatol

Antiphospholipid antibodies in the connective tissue diseases: their relation to the antiphospholipid syndrome and forme fruste disease

J Rheumatol

Prevalence of anticardiolipin antibodies in juvenile chronic arthritis

Ann Rheum Dis

Clinical study
The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases**