Mixed connective tissue disease (pulmonary manifestations)
Pulmonary manifestations of mixed connective tissue disease can be seen in a wide range (20-85%) of those of mixed connective tissue disease. It can have multiple manifestations.
More commonly described features include:
- an interstitial pneumonitis: 20-65%
- pulmonary fibrosis: 20-65%
- pulmonary hypertension: 10-45%
- pleural effusion: ~ 50%
- a consolidative pattern
Other rare presentations include:
- pulmonary vasculitis
- pulmonary thromboembolism
- aspiration pneumonia
- pulmonary hemorrhage
- pulmonary nodules
- pulmonary cysts
- obstructive airways disease
- mediastinal lymphadenopathy
- diaphragmatic dysfunction
Individual imaging features are nonspecific on their own.
A prerequisite for the diagnosis of mixed connective tissue disease is the presence, in the serum, of high titers of antibodies against uridine-rich RNA-small nuclear ribonucleoprotein (snRNP) .