The objective of this study was to investigate the spectrum and frequency o
f rare AIDS-defining diseases in the Swiss HIV Cohort Study. AIDS-defining
diseases contributing less than 1% to the absolute number of all recorded A
IDS-defining diseases in at least one of five periods (1988-1990, 1991-1992
, 1993-1994, 1995-1996, 1997) were defined as being rare. A total of 9110 H
IV-infected subjects were included in this study. Over the entire 9-year pe
riod, the following rare diseases were diagnosed: progressive multifocal le
ukoencephalopathy (Iz = 138), disseminated cryptococcosis (n = 67), viscera
l herpes simplex disease (n = 66), primary cerebral lymphoma (n = 65), inde
terminate cerebral lesion (n = 50), cryptococcal meningitis (n = 34), Mycob
acterium kansasii disease (n = 32), recurrent Salmonella septicemia (n = 22
), intestinal isosporiasis (n = 21), candidiasis of the trachea, bronchi an
d lungs (n = 19), toxoplasma retinitis (n = 16), disseminated toxoplasmosis
(n = 8), invasive cervical carcinoma (n = 8), extrapulmonary Pneumocystis
disease (n = 5), disseminated histoplasmosis (n = 1) and disseminated cocci
dioidomycosis (n = 1). Rare diseases accounted for 7.3% of all AIDS-definin
g diseases over the entire 9-year period. Physicians should be aware of the
likelihood of a broad spectrum of AIDS-defining diseases in HIV-infected p
atients.