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Bronchiolitis obliterans organizing pneumonia associated with Evans syndrome
1Department of Pneumology, Hospital Ramón y Cajal, Madrid, Spain. lmaiz@hrc.insalud.es
Insights
This case report details a rare association between bronchiolitis obliterans organizing pneumonia (BOOP) and both insulin-dependent diabetes mellitus (IDDM) and Evans syndrome in a child. The patient experienced chronic relapses and developed restrictive lung disease despite treatment.
Area of Science:
- Pediatric pulmonology
- Endocrinology
- Autoimmunology
Background:
- Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare lung disease.
- Insulin-dependent diabetes mellitus (IDDM) and Evans syndrome (autoimmune pancytopenia) are distinct conditions.
- The co-occurrence of BOOP with IDDM and Evans syndrome has not been previously documented.
Observation:
- A 4-year-old child with diagnosed IDDM and Evans syndrome presented with prolonged malaise, fever, and nonproductive cough.
- Chest radiography showed bilateral, peripheral patchy alveolar opacities and hilar/mediastinal adenopathy.
- Open lung biopsy confirmed the diagnosis of BOOP.
Findings:
- The patient experienced chronic relapses of BOOP over a 7-year follow-up period.
- Corticosteroid treatment was administered but did not prevent disease recurrence.
- The child ultimately developed restrictive lung disease.
Implications:
- This case highlights a potential, previously unreported association between BOOP, IDDM, and Evans syndrome in pediatric patients.
- Further research is warranted to understand the underlying mechanisms and clinical significance of this triad.
- Recognizing this association may aid in earlier diagnosis and management of complex pediatric respiratory cases.
Abstract:
The association of bronchiolitis obliterans organizing pneumonia (BOOP) with insulin-dependent diabetes mellitus (IDDM) and Evans syndrome (autoimmune pancytopenia) has not been reported previously. We describe the case of a 4-year-old child diagnosed with IDDM and Evans syndrome who presented malaise, fever and nonproductive cough for several months. The chest radiograph revealed several patchy alveolar opacities with peripheral and bilateral distribution and multiple hilar and mediastinal adenopathies. An open lung biopsy established the diagnosis of BOOP. During the follow-up over the next 7 years, the patient had chronic relapses in spite of corticosteroid treatment and developed restrictive lung disease.