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Related Concept Videos

Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

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Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum...
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Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

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Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
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Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

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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.
There are various classifications for PH, each relating to different underlying causes and also...
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Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

842
The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
842
Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

81
Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
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Related Experiment Video

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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[Severe pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV)].

Mohammed Raoufi1, Mustapha Laine1, Hicham Naji Amrani1

  • 1Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.

The Pan African Medical Journal
|February 4, 2017
PubMed
Summary

Pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV) is rare and serious. Aerosol therapy significantly improved a patient with severe obstructive lung disease, offering new therapeutic hope.

Keywords:
Mac Duffiedyspneahypocomplementemia

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Immunology

Background:

  • Hypocomplementemic urticarial vasculitis (HUV), also known as Mac Duffie syndrome, is a rare autoimmune disorder.
  • Pulmonary involvement in HUV is exceptionally uncommon and associated with a poor prognosis.

Observation:

  • A 55-year-old female patient with a 20-year history of HUV presented with severe obstructive pulmonary disease (NYHA class III dyspnea).
  • Diagnosis was confirmed by urticarial lesions, ocular inflammation, low C1q levels, and a positive C1q-p immunodiffusion test.

Findings:

  • Systemic treatment with cyclophosphamide, corticoids, and rituximab showed limited improvement in the patient's severe obstructive pulmonary disease.
  • Initiation of aerosol therapy led to marked clinical improvement in respiratory symptoms and function.

Implications:

  • Pulmonary involvement in HUV significantly worsens the short-term prognosis.
  • Understanding diverse pulmonary manifestations in HUV may reveal novel therapeutic strategies, including aerosol therapy.