What are the three most important vasculitidies that affect the lung?
Wegener granulomatosis, Churg-Strauss granulomatosis and necrotizing sarcoid granulomatosis
A patient presents with cough, hemoptysis, and pleuritis. A lung biopsy reveals large areas of necrosis with a geographical pattern with serpiginous borders and a basophilic center. What is the likely Dx?
What vessels are typically affected in Wegeners Granulomatosis?
Small and medium sized vessels
What other systems are typically affected in Wegeners Granulomatosis?
Kidneys, Head and Neck manifestations (sinusitis, nasal disease)
What is a medical pulmonary emergency associated with WG?
Diffuse Pulmonary Hemorrhage and acute renal failure
What is the typical serum marker associated with WG?
A patient presents with allergic rhinitis, asthma, peripheral eosinophilia and eosinophilic infiltrative disease. A lung biopsy shows a dense infiltrate of chronic inflammatory cells and eosinophils. What is your Dx? What test would be useful in making this Dx? Cause of disease?
Churg-Strauss Disease; P-ANCA; Likely autoimmune
What are the three defining characteristics of Churg-Strauss disease?
Asthma, Eosinophilia and Vasculitis
What are the three stages of Churg-Strauss Disease?
- Prodrome - allergic rhinitis, asthma, peripheral eosinophilia and eosinophilic infiltrative disease
- Systemic Vasculitic Phase: Extrapulmonary vasculitic manifestations (Cutaneous leukocytoclastic vasculitis or peripheral neuropathy)
- Postvasculitic Phase: Cardiovascular manifestations, renal, and sinus involvement
The CXR of an asymptomatic patient shows multiple well-circumscribed, pulmonary nodules. A lung biopsy shows a large area of necrosis surrounded by confluent sarcoidal granulomas. Giant cells along with chronic inflammation featuring lymphocytes and plasma cells are observed. What is the Dx? What is the Tx? What is the Px?
Necrotizing Sarcoid Granulomatosis; Corticosteroids; Excellent