ADOPTING ORPHAN DISEASES RARE INTERSTITIAL LUNG DISEASES

adoptingorphandiseaseskapak

ACUTE SILICOSIS/SILICOPROTEINOSIS

Metin Akgün

Ağrı İbrahim Çeçen University, Faculty of Medicine, Department of Chest Diseases, Ağrı, Türkiye

Akgun M. Acute Silicosis/ Silicoproteinosis. In: Altinisik G, McCormack FX, editors. Adopting Orphan Diseases: Rare Interstitial Lung Diseases. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.61-73.

ABSTRACT

Acute silicosis is a rare and rapidly progressive lung disease that develops after intense, short-term exposure to respirable crystalline silica. Acute silicoproteinosis, a severe subtype, is defined by diffuse intra alveolar accumulation of lipoproteinaceous material and clinically mimics pulmonary alveolar proteinosis (PAP); recent outbreaks in denim sandblasting and engineered stone fabrication underscore its current relevance and its predilection for young workers who may progress to respiratory failure within months. Pathogenesis centers on silica induced injury to alveolar macrophages, with impaired surfactant clearance, activation of the NLRP3 inflammasome and neutrophilic alveolitis, and early profibrotic pathway activation.

Clinically, patients develop rapidly worsening exertional dyspnea that may advance to rest dyspnea, together with dry cough, weight loss, fatigue, and hypoxemia; pulmonary function testing usually shows restriction with reduced diffusing capacity. Diagnosis integrates exposure history with high resolution chest CT showing a characteristic crazy paving pattern and bronchoalveolar lavage yielding opaque, milky fluid rich in PAS positive surfactant material; HRCT may also show air-space consolidations and centrilobular nodules, while hilar or mediastinal lymphadenopathy with eggshell calcification favors silica related disease. Microbiologic evaluation, including testing for tuberculosis, is warranted because silica exposure increases susceptibility to infection.

Management begins with immediate and permanent removal from further silica exposure, followed by supportive care-oxygen supplementation, pulmonary rehabilitation, and vigilant infection prevention and treatment. Whole lung lavage can improve oxygenation and symptoms in selected patients with heavy alveolar filling, while corticosteroids are used empirically with inconsistent, often transient benefit; in progressive or end stage disease, lung transplantation may be the only life saving option. Even after exposure cessation, retained silica can perpetuate inflammation and fibrogenesis, and whole lung lavage generally does not halt disease progression. Thus, overall prognosis remains poor in the absence of curative therapy. Investigational approaches-GM CSF supplementation to restore macrophage function, antifibrotic agents to slow scarring, targeted anti inflammatory therapies, statins, and mesenchymal stem cell-based interventions-are promising but not yet standard. Prevention through stringent exposure control and proactive medical surveillance remains the most effective strategy.

Keywords: Acute silicosis; Silicoproteinosis; Pulmonary alveolar proteinosis; Lung disease; Silica exposure; Whole lung lavage; Occupational diseases

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