RationaleBronchial artery dilatation (BAD) is associated with haemoptysis in advanced cystic fibrosis (CF) lung disease. Our aim was to evaluate BAD onset and its association with disease severity by magnetic resonance imaging (MRI).Materials and Methods188 CF patients (mean age 13.8±10.6 y, range 1.1–55.2 y) underwent annual chest MRI (median three exams, range one to six), contributing a total of 485 MRI examinations incl. perfusion MRI. Presence of BAD was evaluated by two radiologists in consensus. Disease severity was assessed using the validated MRI score and spirometry (ppFEV1).ResultsMRI demonstrated BAD in 71 (37.8%) CF patients consistently from the first available examination, and further ten (5.3%) patients first developed BAD during surveillance. Mean MRI global score in patients with BAD was 24.5±8.3 compared to 11.8±7.0 without BAD (p<0.001), and ppFEV1was lower in BAD compared to patients without (60.8versus82.0%, respectively, p<0.001). BAD was more prevalent in patients with chronicP. aeruginosainfectionversusin patients without (63.6%versus28.0%, p<0.001). In the ten patients who newly developed BAD the MRI global score increased from 15.1±7.8 before to 22.0±5.4 at first detection of BAD (p<0.05). Youden indices for presence of BAD were 0.57 for age (cut-off 11.2 y), 0.65 for ppFEV1(cut-off 74.2%), and 0.62 for MRI global score (cut-off 15.5) (p<0.001).ConclusionMRI detects BAD in patients with CF without radiation exposure. Onset of BAD is associated with increased MRI scores, worse lung function, and chronicP. aeruginosainfection, and may serve as a marker of disease severity.

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Diffuse pulmonary lymphangiomatosis (DPL), an exceptionally rare disease, mainly occurs in children and young adults of both sexes. Even though DPL is considered to be a benign disease, its prognosis is relatively poor. Because of its rarity, little guidance on diagnosis and treatment is available, which makes working with patients with DPL challenging for clinicians. We present here a case of a young man with DPL in whom treatment with sirolimus and propranolol rapidly achieved positive radiological and clinical effects.