Epidemiological Study of Collagen Disease.

An Analysis by Reviewing Nippon Boken Shuho (the Annual of Pathological Autopsy Cases) in Japan.

Abstract

Epidemiological study of autopsy cases of systemic lupus erythematosus (SLE), systemic sclerosis (SS), poly- and dermato-myositis (PD), and periarteritis nodosa (PN) was done by reviewing The Annual of Pathological Autopsy Cases (Nippon Boken Shuho) in Japan during a 7-year period (1972-1978). The result was summarized as follows . 1) Age and sex distribution of autopsy cases of SLE, SS, PD, and PN were well correlated to those of death cases in Vital Statistics Japan from 1972 until 1978 . 2) The total number of autopsy cases reviewed in these 7 years was 180, 350 (107, 931 male, 71, 256 femme; sex not described in 1, 163). Number of cases of collagen disease was calculated as follows. SLE; 90 males (0.08% in total autopsy cases), 667 females (0.94%), SS; 52 males (0.05%), 174 females (0.24%), PD; 99 males (0 .09%), 139 females (0.20%), PN; 72 males (0.07%), 75 females (0 .11%). 3) SLE was the highest in incidence among four groups. The second one was PD . PN was the lowest in incidence. 4) The female to male ratio was 7.9 for SLE, 4.7 for SS, 1.8 for PD, and 1.04 for PN, respectively. 5) The ratio of incompatibility between clinical and pathological diagnoses was the highest in PN. At autopsy, 35 cases (18 males, 17 females) (50.0%) were diagnosed of PN in 70 cases (39 males, 31 females) which were clinically diagnosed of PN. On the contrary, only 35 cases (18 males, 17 females) 23.8%) were clinically diagnosed of PN in 147 cases (72 males, 75 females) which were pathologically diagnosed of PN at autopsy. This result revealed that it was difficult to obtain an accurate clinical diagnosis of PN. 6) The highest incidence of cancer was found in PD, as a complication among all of four groups of collagen disease. Cancer was detected in 53.5% (53/99 males) and 25 .9% (36/139 females) of cases of PD, respectively. In patients older than 50 years of age cancer was recognized in the autopsies of 65.7% of males and 38.2% of females with PD. Secondly, patients with SS showed 9.6% (5/52 males) and 9.2% (15/174 females), respectively. 7) As side effect of steroid administration, atrophy of adrenals was frequently seen at autopsy, particularly in cases of SLE (19%). Opportunistic infection including miliary tuberculosis and fungus infection, also was found in a high incidence.