CTICasesMethamphetamine-Induced Pulmonary Arterial Hypertension and Restrictive Allograft Syndrome
Imaging findings
CT demonstrates profound mosaic perfusion with striking geographic areas of relative ground-glass hyperdensity representing normal or hyperperfused lung, juxtaposed with hypodense areas showing markedly diminutive vessels representing hypoperfused lung, which is confirmed on expiratory CT to be of vascular origin rather than airway-related air trapping. Post-lung transplant imaging years later shows severe progressive upper-lobe volume loss and pleural-parenchymal fibroelastosis-like changes consistent with restrictive allograft syndrome.
Key takeaways
Methamphetamine abuse is a well-established cause of severe Group 1 pulmonary arterial hypertension, which can present with highly atypical, extensive mosaic perfusion reflecting severe, heterogeneous small-vessel pruning. In this patient, progression of the disease necessitated a double lung transplant, after which he ultimately developed restrictive allograft syndrome (RAS). RAS is a phenotype of chronic lung allograft dysfunction (CLAD) characterized radiographically by upper-lobe-predominant pleural and subpleural fibrosis (pleuroparenchymal fibroelastosis pattern) and carries a significantly worse prognosis than bronchiolitis obliterans syndrome.
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