Pulmonary hypertension in the connective tissue disease pathogenesis : clinical features and therapeutic options in 2006 / by Prof. Ronald Asherson.

Format:

Video

Author/Creator:

Asherson, Ronald A., author.

Language:

English

Subjects (All):

Pulmonary hypertension--Pathophysiology.

Pulmonary hypertension.

Connective tissues--Diseases.

Connective tissues.

Physical Description:

1 videorecording (41 min., 32 sec.) : sound, color

Place of Publication:

London : Henry Stewart Talks Ltd, 2007.

System Details:

video file

Contents:

Introduction

Dramatic new developments in past few years

Pulmonary hypertension response

Pulmonary hypertension (PHT)

Epidemiology and pathophysiology

Diagnostic and treatment challenges

WHO classification of pulmonary hypertension

New York Heart Association classification

WHO organization functional classification 1973

Main difference between the two classifications

Pathogenesis of pulmonary hypertension

Imbalance of vascular effectors

Roles of vascular effectors

Production of fibromuscular changes

PHT genetic and immunological factors

PHT characteristics: summary

The various factors involved in PHT pathogenesis

Role of anti-phospholipid antibodies in PHT

Clue for anti-phospholipid antibodies role in PHT

Pulmonary hypertension and systemic sclerosis

PHT and anti-phospholipid antibodies: conclusions

Role of T cells in PHT pathogenesis

Conditions with CD4 cell defects and PAH

Depletion of CD4 cells and autoimmune disease

Autoimmune aspects of PHT

PHT may precede the connective tissue disease

Some idiopathic HPT patients are ANF positive

Anticentromere antibody

Pulmonary arterial hypertension (PAH) and HIV

Relationship to AIRE gene

APECED is a condition of AIRE loss of function

PAH reported following splenectomy

Clinical manifestations

Signs of pulmonary hypertensions

Regular routine examinations and PHT signs

X-ray finding of PHT

Electrocardiography might alert of PAH presence

Laboratory tests

Predisposing factors in thromboembolic PAH (1)

Predisposing factors in thromboembolic PAH (2)

Diagnosis and severity assessment of PAH

Chronic thromboembolic pulmonary hypertension

Embolism travels up to heart and lungs

Histology seen in the lungs

Histological lesions

Examples of vascular pathology

Intimal proliferation in pulmonary hypertension

Medial hypertrophy of the pulmonary arterial

Pulmonary artery lumen and lymphocyte infiltration

Poor prognostic factors

Causes of death

Prognosis of PAH

Survival of pulmonary hypertension patients

PHT in connective tissue disorders

Form of PHT in connective tissue disorders

PHT type variation in connective tissue diseases

PAH in the different connective tissue disorders

Pulmonary hypertension in SLE

Types of pulmonary hypertension in SLE

PHT in SLE and it's associations

PHT and systemic sclerosis characteristics

Frequency of PAH and fibrosis in SSc patients

PAH from heart involvement

Echocardiogram and diastolic dysfunction

Onset of PAH is often delayed

Screening of patients with SSc for PAH

Use of DLCO

DLCO in patients who developed PAH vs. controls

The ratio FVC% / DLCO% is very helpful

FVC/DLCO ratio between 1.6 - 1.8

Cardiopulmonary findings in limited SSc with PAH

Autoantibodies in PAH cases and controls

Frequency of auto-antibodies in all SSc patients

Echocardiography

Echo may indicate PAH but does not diagnose it

Risk factors for PAH in systemic sclerosis

Making diagnosis of PAH

Right heart catheterization as PAH measurement

Dyspnoea/significant mortality and PAH in SSc

Natural history of elevated PASP in SSc

Incidence of HLA DRw52 gene/DR3/DR6 antigens

Anti-phospholipid antibodies

Rheumatoid arthritis and PHT

Elevations of pulmonary arterial pressures

Sjogren's syndrome and PHT

Treatment

Improved survival for patients with IPAH

Approved therapy for SSc PHT

Treprostinal or inhaled iloprost treatment

Continuous i.v. therapy for SSc PHT

Other therapies for SSc PHT

Comments on PAH therapy

New compounds being currently tested

Supportive therapy

Treatment is dependent on type of lung disease

PHT in diffuse SSc

Summary of PAH in SSc

Acknowledgments.

Notes:

Description based on publisher supplied metadata and other sources.

Retrieved April 7, 2024, from https://hstalks.com/bs/202/.