publication . Article . Other literature type . Part of book or chapter of book . 2012

Pulmonary Arterial Hypertension in Connective Tissue Diseases

Bruno Dino Bodini; Simona Sitia; Livio Tomasoni; Matteo Longhi; Maurizio Turiel;
Open Access
  • Published: 30 Jun 2012 Journal: Cardiology in Review, volume 18, pages 85-88 (issn: 1061-5377, Copyright policy)
  • Publisher: Ovid Technologies (Wolters Kluwer Health)
Pulmonary arterial hypertension (PAH) is a progressive disease caused by a remodeling of precapillary arterioles that leads to a progressive increase in pulmonary vascular resistance and right ventricular failure. PAH is associated with significant morbidity and mortality, despite the advent of specific therapies that target pathobiologic pathways implicated in the disease process [1–3]. PAH is can only be diagnosed by right heart catheterization (RHC) and is defined as a mean pulmonary artery pressure greater than 25 mmHg in the absence of elevation of the pulmonary capillary wedge pressure. PAH includes a heterogeneous group of clinical entities sharing simila...
free text keywords: Cardiology and Cardiovascular Medicine, General Medicine, connective tissue diseases, corticosteroid, doppler echocardiography, mixed connective tissue disease, pulmonary hypertension, Article, Immunology and Allergy, Immunology, Rheumatology, Pulmonary and Respiratory Medicine, pulmonary arterial hypertension, systemic sclerosis, Diseases of the musculoskeletal system, RC925-935, Connective tissue, medicine.anatomical_structure, medicine, Dermatomyositis, medicine.disease, Pathology, medicine.medical_specialty, CTD, business.industry, business, Systemic lupus erythematosus, Pulmonary artery, medicine.artery, Vascular resistance, Mixed connective tissue disease, Connective tissue disease, Heart failure, Pulmonary wedge pressure, Portopulmonary hypertension, Pulmonary hypertension, Cardiology, Cause of death, Internal medicine, Polymyositis, Blood pressure, Complication, Doppler echocardiography, medicine.diagnostic_test, Surgery, Pharmacotherapy, Differential diagnosis, Intensive care medicine, Poor prognosis, Central hemodynamics, lcsh:Immunologic diseases. Allergy, lcsh:RC581-607

1. Tojo T, Akiya K, Torikai K et al. [A nationwide epidemiological survey on the complication rate of pulmonary hypertension in patients with mixed connective tissue disease, systemic lupus erythematosus, systemic sclerosis and polymyositis dermatomyositis]. In: [Annual Report of the Ministry of Health and Welfare, Mixed Connective Tissue Disease Research Committee]. 1999;3-6 (in Japanese).

2. Yoshida S, Fukaya S. [Analysis of frequency and pathology of pulmonary hypertension associated with connective tissue diseases]. In: [Annual Report of the Ministry of Health, Welfare and Labour, Systemic Autoimmune Diseases Research Committee]. 2004;40-3 (in Japanese).

3. Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C. The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of communitybased rheumatologists (the UNCOVER study). Arthritis Rheum 2005;52:2125-32.

4. Simonneau G, Robbins IM, Beghetti M et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54:S43-54.

5. Yoshida S, Fukaya S. [Study on the pulmonary hypertension associated with mixed connective tissue disease]. In: [Annual Report of the Ministry of Health, Welfare, and Labour, Mixed Connective Tissue Disease Research Committee]. 2008;37-40 (in Japanese).

6. Torikai K, Fukaya S, Matsumoto Y et al. [Study on the prognosis of mixed connective tissue disease patients complicated with pulmonary hypertension]. In: [Annual Report of the Ministry of Health and Welfare, Mixed Connective Tissue Disease Research Committee]. 1999;20-3 (in Japanese).

7. Yoshida S, Fukaya S, Kyotani S et al. [Revision of the diagnostic criteria for mixed connective tissue disease associated pulmonary arterial hypertension]. In: [Annual Report of the Ministry of Health, Welfare and Labour, Mixed Connective Tissue Disease Research Committee]. 2011;7-13 (in Japanese).

8. Galie N, Hoeper MM, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009;30:2493-537.

9. Humbert M, Sitborn O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004;351: 1425-36. [OpenAIRE]

10. Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical Therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest 2007;131:1917-28.

11. Barst RJ, Gibbs JSR, Ghofrani HA et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2009;54:S78-84.

12. Sanchez O, Sitbon O, Jaïs X, Simonneau G, Humbert M. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 2006;130:182-9.

13. Yoshida S. [Therapeutic Guideline for MCTD pulmonary hypertension . Clinical Guidelines for Mixed Connective Tissue Disease (3rd edition)]. In: Mimori T (ed). [Ministry of Health, Welfare and Labour, Mixed Connective Tissue Disease Research Committee]. 2011;27-32 (in Japanese).

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