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Acute severe idiopathic lymphoid interstitial pneumonia: A case report
Youssef Lamkouan1, Sandra Dury1,2, Jeanne Marie Perotin1,3
1Department of Pulmonary Medicine.
Medicine
|August 15, 2020
Summary
Lymphoid interstitial pneumonia can present acutely and severely. While rituximab may offer temporary improvement, it does not guarantee long-term survival in this rare pulmonary disorder.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Oncology
Background:
- Lymphoid interstitial pneumonia (LIP) is a rare, benign lymphoproliferative disorder of the lungs.
- It often presents chronically but can be associated with autoimmune conditions, dysgammaglobulinemia, or infections.
Observation:
- A 74-year-old woman presented with acute respiratory failure, hypoxemia, and bilateral infiltrates.
- Initial treatments with antibiotics and diuretics were ineffective.
- Extensive workup excluded cardiac, autoimmune, immunodeficiency, and infectious causes; bronchopulmonary lavage showed lymphocytosis.
Findings:
- Surgical lung biopsy confirmed lymphoid interstitial pneumonia without malignancy.
- High-dose corticosteroids provided only mild, transient improvement.
- Rituximab led to dramatic but temporary clinical and radiological recovery, enabling mechanical ventilation weaning.
Implications:
- Idiopathic LIP can manifest as acute, severe respiratory insufficiency.
- Rituximab may provide a transient therapeutic benefit in severe LIP cases.
- The long-term prognosis for acute, severe idiopathic LIP remains poor despite initial treatment responses.
