Name : Shahram
Family : Kahkueei
Affiliation :Assistant Professor of Radiology
Academic Degree : Radiology Department, Masih Hospital, SBMU
Resident : Zahra Amini
Gender : Male
Age : 74
a 74 y/o man with long lasting history of dyspnea
honey combing is visible in lungs bases with skip area and coarse interstitial reticulation
Discussion (Related Text)
UIP is the most common of the idiopathic interstitial pneumonias. It is likely the result of repetitive injury to the lung. The initial response in the lung is inflammation, which is followed by repair and eventually fibrosis. The pathologic abnormalities seen in UIP represent a spectrum of findings, characterized in the early stage of disease by marked proliferation of macrophages in the alveolar airspaces associated with a mild and uniform thickening of the interstitium by mononuclear cells. Late in the course of disease, the pathologic findings are characterized by thickening of the alveolar interstitium by mononuclear inflammatory cells and fibrous tissue. A distinguishing histologic feature of UIP is that different stages of the disease are seen simultaneously within different portions of the lung (temporal heterogeneity). Patients with UIP typically present in the fifth to seventh decades, with a slight male preponderance. Presenting symptoms include progressive dyspnea or a nonproductive cough. Pulmonary function tests show restrictive disease and a decreased diffusing capacity for carbon monoxide (DLCO). Most cases of UIP are idiopathic, but up to 30% of patients with UIP have an associated collagen vascular or immunologic disorder. This is most often rheumatoid arthritis, but it can also be SLE, scleroderma, or dermatomyositis/polymyositis. The radiographic manifestations of UIP parallel the pathologic changes. In the early phase of disease, the chest radiograph may appear normal despite the presence of clinical symptoms and abnormalities on pulmonary function testing. The earliest radiographic changes are bibasilar fine to medium reticular opacities or ground glass density. As the disease progresses, a coarse reticular or reticulonodular pattern is seen, which almost invariably leads to the formation of honeycomb cysts (3 to 10 mm in diameter) and progressive loss of lung volume. Extensive pulmonary fibrosis may be associated with findings of pulmonary arterial hypertension.
Brant WE, Helms CA: Fundamentals of Diagnostic Radiology, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 2012.