PT  - JOURNAL ARTICLE
AU  - Shinobu Akagawa
AU  - Ryota Sato
AU  - Kennichi Okuda
AU  - Kazuya Koyama
AU  - Masahiro Shimada
AU  - Kimihiko Masuda
AU  - Hirotosi Matsui
AU  - Ken Ohta
TI  - Three cases of idiopathic pleuroparenchymal fibroelastosis associated with amyotrophic lateral sclerosis
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P3748
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P3748.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P3748.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Introduction: In Japanese cases of idiopathic pleuroparenchymal fibroelastosis (IPPFE), radiological abnormalities are mostly localized in the bilateral upper lobes. With disease progression, patients may complain severe dyspnea and lose much weight like neuromuscular diseases. We reported and discussed about the clinical features of 3 patients clinically diagnosed as IPPFE associated with amyotrophic lateral sclerosis (ALS).Report: At the time of diagnosis of IPPFE, patients were 49-yr-old (male, Case 1), 53-yr-old (female, Case 2), and 63-yr-old (male, Case 3). Symptoms including severe dyspnea, tachypnea, severe atrophy around upper chest wall, dysphagia, articulation disorder appeared 2 years after the diagnosis in Case 1, 4 years in Case 2, and 7 years in Case 3. As they had no muscular dysfunctions in extremities at the time, diagnosis of ALS was denied in consultation with neurologists then in spite of the bulbar palsy. Diagnosis of ALS was confirmed when their symptoms and signs progressed 6 months after in Case 1, 2 years in Case 2, and 6 months in Case 3. At the onset of ALS, PaCO2 / PaO2 were 50 torr / 92 torr in Case 1, 48/ 82 in Case 2, and 50 / 98 in Case 3. Body mass indices at the onset of IPPFE were 14.4 in Case 1, 17.2 in Case 2, and 18.8 in Case 3, and they were further decreased at the onset of ALS.Discussion: All 3 patients of IPPFE associated with ALS presented initial symptoms caused from weakness of respiratory muscles and bulbar palsy which are not typical in the early stage of ALS. A patient of IPPFE who exhibits bulbar palsy and severe dyspneic sensation inappropriate for pulmonary lesion should be suspected of the association with ALS.