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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Lymphoid interstitial pneumonia associated with common variable immunoglobulin deficiency
Mikako Matsubara1, Tomonobu Koizumi, Toshihide Wakamatsu
1The First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto. mtsunoda@hsp.md.shinshu-u.ac.jp
Internal Medicine (Tokyo, Japan)
|April 19, 2008
Summary
A 30-year-old woman with common variable immunodeficiency was diagnosed with lymphoid interstitial pneumonia after abnormal chest imaging. Treatment with prednisolone and immunoglobulin stabilized her condition.
Area of Science:
- Pulmonology
- Immunology
- Pathology
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low immunoglobulin levels and impaired B-cell function.
- Patients with CVID are susceptible to recurrent infections and autoimmune phenomena, but pulmonary complications are less common.
- This case highlights an unusual pulmonary manifestation in a CVID patient.
Observation:
- A 30-year-old woman with known CVID presented with incidental abnormal findings on chest radiographs during a routine health screening.
- Chest computed tomography (CT) revealed multiple reticulonodular infiltrates predominantly in the lower lung fields.
- Surgical lung biopsy was performed for definitive diagnosis.
Findings:
- Histopathological examination of the lung biopsy demonstrated a diffuse infiltration of mature small lymphocytes and other mononuclear cells within the lung parenchyma and along the bronchiolar walls.
- These findings were consistent with a diagnosis of lymphoid interstitial pneumonia (LIP).
- No evidence of infection or malignancy was identified.
Implications:
- Lymphoid interstitial pneumonia (LIP) can be a rare pulmonary manifestation of common variable immunodeficiency (CVID).
- Early diagnosis and appropriate treatment, including corticosteroids and immunoglobulin replacement therapy, can lead to clinical and radiographic stabilization.
- This case underscores the importance of considering uncommon pulmonary conditions in immunodeficient patients presenting with respiratory abnormalities.
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