Tafamidis meglumine treatment was associated with radiologic stability of pulmonary ground-glass opacities over one year in an 80-year-old patient with systemic transthyretin amyloidosis and pulmonary-dominant presentation.
Case Report (n=1)
No
Does tafamidis stabilize pulmonary lesions in a patient with pulmonary-dominant ATTR amyloidosis?
Tafamidis therapy may stabilize pulmonary lesions in patients with pulmonary-dominant ATTR amyloidosis, though longer observation is needed.
Pulmonary-dominant transthyretin (TTR) amyloidosis is an uncommon condition. Its presentation with persistent ground-glass opacity (GGO) and infiltrates mimics common respiratory diseases, causing diagnostic delays. Here, we report a case of an 80-year-old male patient with a five-year history of GGO refractory to antibiotics and steroids. Endobronchial ultrasound-guided transbronchial lung biopsy confirmed TTR amyloidosis. Investigations revealed subclinical cardiac involvement, supporting systemic amyloid TTR (ATTR) with a pulmonary-dominant phenotype. Treatment with tafamidis was initiated; however, pulmonary lesions remained radiologically stable at two months and at one year of follow-up. Given that tafamidis is expected to stabilize TTR and slow disease progression rather than rapidly regress established deposits, short-term radiologic stability should be interpreted cautiously. This case demonstrates the diagnostic utility of minimally invasive bronchoscopic techniques for unexplained lung opacities and highlights the need for further evidence regarding the clinical course and treatment response of pulmonary manifestations of ATTR.
Uchida et al. (Wed,) conducted a case report in 80-year-old male patient with systemic transthyretin amyloidosis presenting with pulmonary-dominant manifestations including persistent ground-glass opacities and subclinical cardiac involvement (n=1). Tafamidis meglumine was evaluated on Radiologic stability of pulmonary opacities (ground-glass opacities and nodules) assessed by chest CT over one year of tafamidis therapy. Tafamidis meglumine treatment was associated with radiologic stability of pulmonary ground-glass opacities over one year in an 80-year-old patient with systemic transthyretin amyloidosis and pulmonary-dominant presentation.