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VASCULITIS CAUSING BRONCHIAL ASTHMA
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07.14.08 (3 months ago)
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Rubin’s Pathology
– Clinicopathological Foundations of Medicine - 5th Ed
Ch 13 – The Respiratory System
Wegener Granulomatosis Affects the Respiratory Tract and Kidneys
WG is a disease of unknown cause characterized by aseptic, necrotizing, granulomatous inflammation, and vasculitis that affect both upper and lower respiratory tracts, and the kidneys.
Clinical Features
WG most commonly affects the head and neck, followed by the lung, kidney, and eye. Respiratory manifestations include cough, hemoptysis, and pleuritis. Chest radiographs commonly show multiple intrapulmonary nodules, although single nodules may also be encountered.
Diffuse pulmonary hemorrhage,an important complication of WG, is a fulminant life-threatening crisis characterized by severe respiratory failure. It is usually accompanied by acute renal failure.
Ch 10 – The Blood Vessels
Polyarteritis Nodosa Is an Acute, Necrotizing Vasculitis
As a result of thrombosis in affected segment of an artery, infarcts are commonly found in involved organs. Injury to larger arteries causes small aneurysms (<0.5 cm in diameter), particularly in branches of the renal, coronary, and cerebral arteries. An aneurysm may rupture and, if located in a critical area, may result in fatal hemorrhage.
Clinical manifestations of polyarteritis nodosa are highly variable, and depend on the chance locations of lesions in different organs. Kidneys, heart, skeletal muscle, skin, and mesentery are most frequently involved, but lesions may occur in almost any organ, including the bowel, pancreas, lungs, liver, and brain.
Hypersensitivity Angiitis Is a Response to Exogenous Substances
Systemic hypersensitivity angiitis, also referred to as microscopic polyarteritis, affects many of the same organs as polyarteritis nodosa but is restricted to the smallest arteries and arterioles.
The disease typically presents as palpable purpura, principally on the lower extremities. Microscopically, superficial cutaneous venules display fibrinoid necrosis with acute inflammatory reaction. Cutaneous vasculitis is generally self-limited.
Churg-Strauss Syndrome (Allergic Angiitis & Granulomatosis) is defined by Asthma, Eosinophilia & Vasculitis
Pathology
The lungs of patients with Churg-Strauss syndrome show changes of asthmatic bronchitis or bronchiolitis
Churg-Strauss syndrome passes through three clinical phases.
Prodrome: Patients have one or more of: allergic rhinitis, asthma, peripheral eosinophilia, and eosinophilic infiltrative disease (eosinophilic pneumonia or eosinophilic enteritis).
Systemic vasculitic phase: Extrapulmonary vasculitic manifestations are present, such as cutaneous leukocytoclastic vasculitis or peripheral neuropathy.
Postvasculitic phase: Patients may continue to have asthma and allergic rhinitis, and complications of neuropathy and hypertension may persist. Cardiovascular manifestations are common and often consist of pericarditis, hypertension, and cardiac failure. Renal disease and sinus involvement are usually less severe than those in WG.
Harrison's Internal Medicine > Part 13. Disorders of the Immune System, Connective Tissue, and Joints > Section 2. Disorders of Immune-Mediated Injury > Chapter 319. The Vasculitis Syndromes >
Wegener's Granulomatosis
Involvement of the upper airways occurs in 95% of patients with Wegener's granulomatosis. Patients often present with severe upper respiratory tract findings such as paranasal sinus pain and drainage and purulent or bloody nasal discharge, with or without nasal mucosal ulceration
Pulmonary involvement may be manifested as asymptomatic infiltrates or may be clinically expressed as cough, hemoptysis, dyspnea, and chest discomfort. It is present in 85–90% of patients. Endobronchial disease, either in its active form or as a result of fibrous scarring, may lead to obstruction with atelectasis.
Churg-Strauss Syndrome
Definition
Churg-Strauss syndrome, also referred to as allergic angiitis and granulomatosis, was described in 1951 by Churg and Strauss and is characterized by asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, and vasculitis of multiple organ systems.
The pulmonary findings in Churg-Strauss syndrome clearly dominate the clinical picture with severe asthmatic attacks and the presence of pulmonary infiltrates.
Harrison's Internal Medicine > Chapter 248. Asthma
Differential Diagnosis
Eosinophilic pneumonias and systemic vasculitis, including Churg-Strauss syndrome and polyarteritis nodosa, may be associated with wheezing.
Hence, it is only in Churg-Strauss Syndrome that vasculitis is accompanied by bronchial asthma. The other conditions find no direct mention of asthma. Only PAN is mentioned as a DD of wheezing by Harrison.
I will go with Churg-Strauss Syndrome as the correct answer.
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