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Joseph Junewick, MD FACR
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Alveolar Proteinosis

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Infectious-Inflammatory
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: CT
Activities:
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History

9 year old female with cough and history of “recurrent pneumonias”.


Case Images


Diagnosis

Alveolar Proteinosis

Clinical Notes

Laboratory – positive p-ANCA

Pathology – Wedge biopsy revealed alveolar proteinosis

Findings

CR – Geographic central parahilar opacities.

CT – Sharply marginated geographic zones of alveolar opacitfication and scattered linear septal thickening with relative peripheral sparing.

Discussion

Pulmonary alveolar proteinosis is a rare disease characterized by abnormal intraalveolar accumulation of surfactant-like material. Three distinct subgroups of alveolar proteinosis are currently recognized: idiopathic, secondary, and congenital. Idiopathic accounts for approximately 90% of cases. This form occurs worldwide, with an incidence of 0.36 new cases per 1 million persons each year. Secondary alveolar proteinosis occurs in patients with industrial inhalational exposure (e.g., silica particles, cement dust, aluminum dust, titanium dioxide, nitrogen dioxide, and fiberglass), underlying hematologic malignancy, or immunodeficiency disorders (immunosuppressive therapy and human immunodeficiency virus infection). Congenital alveolar proteinosis is quite rare and manifests in the neonatal period with severe hypoxia.

The common pathophysiology of these forms of alveolar proteinosis appears to be related to pulmonary surfactant and lung immune function, specifically deficiency of surfactant proteins or impaired function of granulocyte-macrophage colony-stimulating factor which is an essential regulator of immune defense and surfactant homeostasis.

Chest radiography typically reveals bilateral central and symmetric lung opacities, with relative sparing of the apices and costophrenic angles. The CT appearance is characterized as “crazy-paving” with smoothly thickened septal lines superimposed on areas of ground-glass opacity. Areas of crazy-paving are typically widespread and bilateral, often with sharply marginated areas of geographic or lobular sparing. Although the CT finding of crazy-paving is highly characteristic of alveolar proteinosis, it is also seen in infection, hemorrhage, neoplasia, inhalational disease, and hydrostatic pulmonary edema.

Reference

Frazier AA, et al. Pulmonary Alveolar Proteinosis. Radiographics 2008; 28:883-899.

Contributor

Eric Payne, MD



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