Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


Determination of the Accurate Incidence of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis, Mixed Connective Tissue Disease and Systemic Lupus Erythematosus by Prospective Study for 3-Years.

Tanaka1,  Sumiaki, Hoshi4,  Kenta, Tanaka4,  Junichi, Wada4,  Tatsuhiko, Matsui4,  Toshimichi, Nagai4,  Tatsuo, Okada2,  Jun

Drexel University College of MedicineDepartment of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan
Drexel University College of MedicineDepartment of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Sagamihara, Japan
Drexel University College of Medicine Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Kanagawa, Japan
Drexel University College of MedicineDepartment of Rheumatology and Infectious Diseases, Kitasato University School of Medicine

Background:

Connective tissue diseases (CTDs) are susceptible to pulmonary arterial hypertension (PAH), which has not been well evaluated.

Objective:

To clarify the accurate incidence of PAH in patients with diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD).

Patients and Methods:

Prospective study of the 451 consecutive Japanese patients with dcSSc, lcSSc, SLE and MCTD enrolled from Nov 2006 until Mar 2010. All these patients had not been diagnosed of PAH before this study. The diagnosis of PAH was performed based on estimated systolic pulmonary arterial pressure (PAP) >40 mmHg with findings of right ventricular pressure and/or volume overload using ultrasound cardiography (UCG), followed by confirmation using right heart catheterization. Incidence of PAH (per 1000 person-years) was estimated using a generalized linear model (Poisson distribution, link function: log).

Results:

451 patients consisted of 75 dcSSc, 74 lcSSc, 230 SLE, and 82 MCTD patients. 8 patients were excluded due to the presence of PAH at the enrollment (Figure 1).

Thus 443 patients were enrolled and followed during 373 patient-years. Among the 443 patients, 11 patients were suspected to have PAH by UCG, and 5 of the 11 patients (2 dcSSc, 1 lcSSc, 1 SLE, 1 MCTD) were diagnosed as definite PAH. The incidence of PAH was significantly high in dcSSc and low in SLE (Table 1 and Figure 2).

Conclusions:

These results have disclosed the exact incidence of PAH in patients with CTDs, which was strikingly higher than that of idiopathic PAH (~2–3 per million person-years). The data also indicate that the incidence of PAH in dcSSc is much higher than that in MCTD and lcSSc.

Figure 1. Follow-up Chart of Study PAH: pulmonary arterial hypertension, VSD: ventricular septum defect, LHF: left heart failure, RHC: right heart catheterization

Figure 2. Incidences of PAH in various CTDs.

Table 1. Incidences of PAH in patients with various CTDs

CTDsestimated averages95% confidence interval
*dcSSc26.8322.83–31.51
lcSSc14.8711.81–18.74
*SLE6.225.46–7.08
MCTD11.829.50–14.72
Incidence:/person-years, *p < 0.0001

To cite this abstract, please use the following information:
Tanaka, Sumiaki, Hoshi, Kenta, Tanaka, Junichi, Wada, Tatsuhiko, Matsui, Toshimichi, Nagai, Tatsuo, et al; Determination of the Accurate Incidence of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis, Mixed Connective Tissue Disease and Systemic Lupus Erythematosus by Prospective Study for 3-Years. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :568
DOI: 10.1002/art.28337

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