1. Analytical studies were performed on the roentgenographic manifestations in 22 P.S.S. cases (non-treated, 22 female, average age 38).
Chest roentgenographic manifestations were expressed by scores calculated from roentgenographic changes classified into 11 pattern categories, i.e. ground glass pattern (a), fine mottled pattern (b) mottled pattern (c), cloudy shadow (d), line shadow (L), fine reticular pattern (X), medium reticular pattern (Y), coarse reticular pattern (Z), honeycombing (H), reduction of lower lung field (F) and elevation of diaphragm (D). The densities and extent of these patterns were classified into 3 grades, the numbers were multiplied by each other, the products were summed up and total scores were calculated. These scores express the degree of roentgenographic manifestations in each patient and were also compared in terms of age, duration of disease and pulmonary function test changes (%VC, and DLco).
2. Ground glass patterns (a) were often observed in early stage patients with severe lung involvement (1st group and 2nd group, 5 cases) (Tab 1). In these cases, densities and extent of the patterns were greater (a3×III-a2×III) and total scores usually amounted to more than 10. %VC and DLco were also remarkably reduced. Mottled patterns (b) were observed also in early stage cases but with only slight lung involvement (13rd group, 4th group, 10cases). In these cases density and extent were low grade (b1×I-b1×II) and total scores usually amounted to less then 5. %VC was normal but reduction of DLco was always present.
Mottled patterns were equally observed in patients of chronic stage. The duration of disease of this group was always more than 4 years and the average age was 48 years (5th group, 7 cases). In these cases, some of the patterns of fibrotic changes, namely L, X, Y, Z, F, or D were observed synchronously. In 5, total scores were distributed in the 5-10 range and moderate reduction of %VC and DLco advanced very slowly.
3. Using this scoring-method of roentgenographic manifestations, we could divide the time course of lung involvement into 2 different types. The 1st is the fulminating type, becoming rapidly severe (1st and 2nd group). The 2nd remains in an arrested state and detoriorates slowly (5th group). Therefore this scoring-method, it is very useful toforecast the prognosis of lung inolvement particularly at the onset of the disease.
4. In early stage cases with slight lung imvolvment, %VC was always normal but DLco was reduced without exception. Because of good correlation between DLco and roentgenographic scores (Fig. 2), it is advisable to use roentgenographic scores as well as DLco to diagnose slight lung involvment in the early stage of P.S.S..
