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04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Hepatopulmonary syndrome and diffuse interstitial lung disease]
G Tercé1, L Stervinou-Wemeau, S Leroy
1Centre de compétence des maladies pulmonaires rares, service de pneumologie et d'immuno-allergologie, hôpital A.-Calmette, CHRU de Lille, boulevard du Pr-J.-Leclercq, 59037 Lille, France.
Revue Des Maladies Respiratoires
|November 30, 2010
Summary
Idiopathic interstitial pneumonia can be associated with hepatopulmonary syndrome, causing severe hypoxemia. Early diagnosis of intrapulmonary shunting and liver disease is crucial for managing this rare condition.
Area of Science:
- Pulmonology
- Hepatology
- Cardiology
Background:
- The co-occurrence of interstitial pneumonia and hepatopulmonary syndrome (HPS) is infrequently documented.
- Idiopathic interstitial pneumonia (IIP) can present with disproportionately severe hypoxemia.
Observation:
- Two patients with IIP and severe hypoxemia (PaO2 42 and 47 mmHg) were diagnosed with intrapulmonary shunting via 99mTc-MAA lung imaging and contrast echocardiography.
- Both patients had hepatic cirrhosis (Child Grade A6 and C10), leading to the diagnosis of HPS associated with IIP.
Findings:
- Severe hypoxemia in patients with interstitial pneumonia necessitates investigation for underlying hepatopulmonary syndrome.
- Diagnosis of intrapulmonary shunting is confirmed through nuclear medicine lung imaging and cardiac ultrasound.
Implications:
- Liver transplantation is the definitive treatment for the pulmonary manifestations of HPS.
- The rare association of interstitial lung disease (ILD) and HPS complicates transplantation decisions, especially without a lung biopsy and clear prognosis.
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