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Hermansky-Pudlak Syndrome Complicated by Pulmonary Fibrosis

A 45-year-old woman from Puerto Rico presented to the emergency department with progressively worsening dyspnea over a 3- to 4-day period. She had been diagnosed with type 1 Hermansky-Pudlak syndrome complicated by pulmonary fibrosis 4 years earlier. Previous renal biopsy demonstrated nephrosclerosis secondary to Hermansky-Pudlak syndrome. A brother also had Hermansky-Pudlak syndrome complicated by pulmonary fibrosis. Despite receiving 6 L of oxygen via nasal cannula at home, her oxygen saturation had fallen to 80% to 85%. She was subsequently admitted to the intensive care unit and intubated. Broad-spectrum antibiotics were initiated for a presumptive diagnosis of pneumonia. 
 
A chest radiograph obtained at the time of admission showed reticular opacities throughout the bilateral lungs (Figure ​(Figure11). There was no evidence of consolidation. Contrast-enhanced chest computed tomography (CT) demonstrated extensive subpleural honeycombing, consistent with pulmonary fibrosis (Figures ​(Figures22 and ​and33). Patchy ground-glass opacity was also present bilaterally. The patient had been scheduled for lung and kidney transplant but had fatal cardiac arrest in the intensive care unit and died. 
 
 
 
Figure 1 
 
Frontal chest radiograph shows bilateral reticular opacities, greater on the left than the right. 
 
 
 
 
 
Figure 2 
 
Axial CT images through the (a) upper lung zones and (b) lower lung zones demonstrate extensive subpleural honeycombing bilaterally, which is more prominent in the lower lung zones. Ground-glass opacity is also present. 
 
 
 
 
 
Figure 3 
 
Coronal CT image shows a greater degree of honeycombing within the peripheral lung than the central lung and within the mid and lower lung zones than the upper lung zones.