The Lungs in Autoimmune Diseases Flashcards
What three pulmonary abnormalities can be seen in patients with SLE?
Painful pleuritis +/- effusion, diffuse atelectasis, and sometimes diaphragmatic weakness. The diaphragmatic weakness can additionally cause orthopneic dyspnea out of proportion to the CXR findings.
What are the two pulmonary effects associated with systemic scleroderma?
Interstitial fibrosis and intimal proliferation
Which collagen vascular disease causes pulmonary HTN out of proportion to the pulmonary disease noted?
The intimal proliferation associated with systemic scleroderma causes pulmonary HTN out of proportion to the pulmonary disease when it affects the pulmonary artery.
How is the respiratory system affected in patients with granulomatosis with polyangiitis (GPA)?
GPA is a systemic vasculitis with necrotizing granulomas, which affects both the upper respiratory tract (nose and sinuses) and the lower respiratory tract (pulmonary vasculitis).
A patient presents with purulent nasal discharge, epistaxis, and/or signs of glomerulonephritis with hematuria. What condition do you suspect?
Granulomatosis with polyangiitis (GPA). Remember: sinus, lungs, kidneys.
Which laboratory test is positive in many patients with granulomatosis with polyangiitis (GPA)?
c-ANCA is ~90% sensitive and specific for GPA when present.
A patient presents with dyspnea, hemoptysis, iron deficiency anemia, and glomerulonephritis without upper airway involvement. What condition do you suspect?
Antiglomerular basement membrane antibody disease (Goodpasture syndrome).
How might one differentiate between granulomatosis with polyangiitis (GPA), idiopathic pulmonary hemosiderosis (IPH), and antiglomerular basement membrane antibody disease (Goodpasture syndrome) based on presenting symptoms?
Goodpasture would have the same pulmonary symptoms as IPH but with the addition of kidney involvement. In contrast with IPH, patients with Goodpasture will have hemoptysis but will not typically have frank hemorrhage. GPA and Goodpasture both affect the lungs and kidneys in similar ways, but GPA also affects the sinuses, which are not involved in Goodpasture.
What causes the pulmonary and renal issues associated with Goodpasture syndrome (anti-GBM antibody disease)?
Symptoms are due to anti-GBM antibodies, which cause linear deposition of IgG and C3 on alveolar and glomerular basement membranes.
How does one definitively diagnose Goodpasture syndrome?
Renal biopsy demonstrating anti-GBM antibodies.
How does one definitively diagnose granulomatosis with polyangiitis (GPA)?
Diagnosis can be confirmed from either a biopsy of the nasal membrane or an open lung biopsy.
