A case of airway aluminosis with likely secondary pleuroparenchymal fibroelastosis

Abstract

Background Excessive inhalation of aluminium powder occasionally results in upper lobe predominant lung fibrosis, which is similar to idiopathic pleuroparenchymal fibroelastosis (IPPFE) and has been suggested to be secondary PPFE. Case presentation A 67-year-old man who had worked in an aluminum-processing factory for 50 years visited our hospital complaining of exertional dyspnea. Chest computed tomography (CT) showed bilateral dense sub-pleural consolidation in the upper and middle lung fields, which was consistent with IPPFE; however, the possibility of secondary PPFE associated with aluminosis was not ruled out. Considering the patient’s critical condition, trans-bronchial lung biopsy (TBLB) rather than surgical lung biopsy was performed, with elemental analysis of the biopsied specimen. Unfortunately, the specimen obtained by TBLB did not contain alveolar tissue; therefore, pathological diagnosis of PPFE was not possible. However, radiographic findings were highly suggestive of PPFE. On elemental analysis, excessive amounts of aluminum were detected in the bronchiolar walls, establishing a diagnosis of airway aluminosis with likely secondary PPFE resulting from aluminium exposure. Conclusions TBLB with elemental analysis might be useful in differentiating idiopathic PPFE from secondary causes in dust inhalation related disease, such as aluminosis. This case indicated that inhalation of aluminium might cause secondary PPFE, with attention needing to be paid to avoid further exposure.

Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE)

“…Several recent studies have explored and documented the possible etiologies of ondary PPFE, including radiation [27], bone marrow or stem cell transplantation [28 lung transplantation [28,30], connective tissue diseases (CTDs) [31][32][33], asbestosis pneumoconiosis [34,35], metal lung [36,37], recurrent infections (e.g., pulmo Along with the progressive disease course, diminution of the upper lobes causes volume loss and decreased range of motion of the rib cage and diaphragm, which increases end-expiratory air trapping. Mainly, dyspnea due to dominant upper-lobe volume loss affects the "effort to breathe", whereas air trapping and decreased range of motion of the rib cage and diaphragm lead to dyspnea experienced as "air hunger".…”

“…Several recent studies have explored and documented the possible etiologies of secondary PPFE, including radiation [27], bone marrow or stem cell transplantation [28,29], lung transplantation [28,30], connective tissue diseases (CTDs) [31][32][33], asbestosis [34], pneumoconiosis [34,35], metal lung [36,37], recurrent infections (e.g., pulmonary aspergillus [4] or Mycobacterium avium complex [38]), chemotherapeutic agents [39,40], and fibrotic hypersensitivity pneumonitis (fHP) [4,41,42]. Based on a report by Oda et al, fHP was relatively common in the secondary PPFE group [14].…”

Enfermedades del intersticio pulmonar de causa laboral. (Parte I)

“…Si bien este metal, en general, produce EPID OCUP con caracteres de IPF, los mecanismos de reparación del parénquima pulmonar manifiestan una sensibilidad individual que ocasiona esta respuesta. [58][59][60]…”

Pleuroparenchymal fibroelastosis

“…Rare genetic variants, especially telomere-related gene mutations, were identified in patients with PPFE, some of them with a family history of ILD [20,21]. Similarly, a pattern of PPFE in the upper lung zones may be associated with a usual interstitial pneumonia (UIP) pattern in the Environmental exposures (asbestosis, silica, aluminum) [45,46] Familial or genetic disease (telomeropathy) [20,21] Liver transplantation [47] lung bases [22-24,…”