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Pulmonary Hypertension: Classification and Pathogenesis01:30

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Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
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The systemic and pulmonary circuits are crucial components of the circulatory system, working together to transport blood between the heart, lungs, and the rest of the body. The process begins with pulmonary circulation, where deoxygenated blood is pumped from the right ventricle to the lungs via the pulmonary trunk and arteries. Upon reaching the lungs, the blood becomes oxygenated and returns to the heart, specifically to the left atrium, via the pulmonary veins.
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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Pulmonary involvement in primary systemic vasculitides.

Jean-Paul Makhzoum1, Peter C Grayson2, Cristina Ponte3,4

  • 1Vasculitis Clinic, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal, Montreal, QC, Canada.

Rheumatology (Oxford, England)
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Summary

Pulmonary manifestations occur in all primary systemic vasculitides, particularly ANCA-associated vasculitis (AAV). These lung issues can lead to lasting damage, especially in AAV patients.

Keywords:
ANCA-associated vasculitisinterstitial lung diseasepulmonaryvasculitis

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Internal Medicine

Background:

  • Primary systemic vasculitides are a group of rare autoimmune diseases.
  • Pulmonary involvement is a known but variably reported complication across different vasculitis types.

Purpose of the Study:

  • To characterize the spectrum and initial impact of pulmonary manifestations in primary systemic vasculitides.
  • To compare pulmonary involvement across various vasculitis subtypes.

Main Methods:

  • Retrospective analysis of data from 1952 patients with primary systemic vasculitides.
  • Inclusion of patients with Takayasu's arteritis (TAK), GCA, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), polyarteritis nodosa (PAN), and IgA vasculitis (IgAV).
  • Assessment of pulmonary symptoms, imaging findings, mechanical ventilation needs, and long-term damage using the Vasculitis Damage Index.

Main Results:

  • Pulmonary manifestations were observed in 21.8% of TAK, 15.8% of GCA, 64.5% of GPA, 65.9% of MPA, 89.0% of EGPA, 27.1% of PAN, and 5.9% of IgAV patients.
  • Dyspnea and cough were common symptoms, with higher prevalence in EGPA, MPA, and GPA.
  • Haemoptysis was mainly seen in ANCA-associated vasculitis (AAV).
  • Pulmonary fibrosis was noted in MPA (24.9%) and EGPA (6.3%).
  • Mechanical ventilation was required only in AAV patients (GPA, MPA, EGPA).
  • At 6 months, 52.7% of EGPA patients had pulmonary damage in the Vasculitis Damage Index, followed by MPA (28.7%) and GPA (15.4%).

Conclusions:

  • Pulmonary manifestations are a significant feature across all primary systemic vasculitides.
  • ANCA-associated vasculitis (AAV) subtypes (GPA, MPA, EGPA) exhibit higher frequencies of pulmonary involvement and are more prone to developing permanent lung damage.