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Updated: May 10, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary alveolar microlithiasis with minimal symptoms and near-complete whiteout on chest imaging
Nam Nguyen-Thanh1, Ngoc Duong-Minh1,2, Khoa Nguyen-Dang1,2
1Department of Pulmonary Medicine, Cho Ray Hospital, Ho Chi Minh City, Vietnam.
Abstract:
Pulmonary alveolar microlithiasis (PAM) is a rare idiopathic disease characterized by accumulated calcium and phosphate crystals within the alveoli. Although PAM can be suspected in patients with clinical-radiological dissociation and characteristic imaging findings on chest computed tomography, definitive diagnosis requires a family history of PAM, identification of SLC34A2 gene mutations, or lung biopsy to exclude differential diagnoses. We report a case of a 66-year-old female incidentally found to have diffuse pulmonary calcifications. The diagnosis was confirmed through typical imaging features, transbronchial lung biopsy, and a family history of PAM. This case highlights the hallmark imaging characteristics of PAM and the critical role of transbronchial lung biopsy in establishing a definitive diagnosis. As PAM is a rare disease with no established consensus on treatment, except for lung transplantation, symptomatic management remains a significant challenge.
Insights
Pulmonary alveolar microlithiasis (PAM) is a rare lung disease. Diagnosis involves imaging, biopsy, and family history, crucial for managing this condition.
Area of Science:
- Pulmonary Medicine
- Rare Diseases
- Medical Imaging
Background:
- Pulmonary alveolar microlithiasis (PAM) is a rare idiopathic interstitial lung disease.
- Characterized by the accumulation of calcium and phosphate crystals within the pulmonary alveoli.
- Diagnosis can be challenging due to its rarity and varied presentation.
Observation:
- A 66-year-old female presented with incidentally discovered diffuse pulmonary calcifications.
- Clinical-radiological dissociation and characteristic chest computed tomography (CT) findings suggested PAM.
- Diagnosis was confirmed by typical imaging, transbronchial lung biopsy, and a positive family history.
Findings:
- Transbronchial lung biopsy is critical for definitive diagnosis of PAM, excluding other conditions.
- The case underscores the importance of integrating imaging, biopsy, and family history for accurate PAM diagnosis.
- Hallmark imaging features on chest CT aid in early suspicion of this rare lung disease.
Implications:
- Definitive diagnosis of PAM relies on a combination of clinical, radiological, and pathological findings.
- Symptomatic management of PAM presents a significant challenge due to the lack of established treatment guidelines beyond lung transplantation.
- Further research into the genetic basis and therapeutic strategies for PAM is warranted.
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