SummaryDue to a short observation period previous studies may have underestimated prevalence and recurrence risk of IDDM in relatives of IDDM patients. To obtain a more exact life-time risk estimate we identified 310 probands, representative of Danish IDDM patients, characterized by current age more than 50 years, age at onset 40 years or less and diabetes duration of more than 30 years. Family data were obtained from 291 probands. Mean "observation" times (age) (+ SD) for siblings (n =553) and offspring (n = 359) were 59.4 + 16.1 years and 33.8 + 8.8 years, respectively. Of the probands 73 (25.1%) had at least one first-degree relative with IDDM. Seventeen percent had at least one affected sibling. An increase from 10.4 % to 22.4 % of having first-degree relatives with IDDM among probands with age at onset below 20 years was observed during the period from proband at age 21 years up to 1 September 1992. Among affected siblings 48 % of the second cases were affected more than 10 years after the first affected sibling. Using the life-table method cumulative recurrence risks from time of birth were calculated for siblings up to age 30 years of 6.4 % and up to age 60 years of 9.6 %. For offspring the risk up to age 34 years was 6.3 %. In addition, we present a life-table method evaluating the cumulative recurrence risk from time of onset in the proband, as this is the most relevant when giving genetic counselling. In conclusion, the long-term risks of IDDM in siblings and offspring are high compared to that shown in previous reports. [ Diabetologia (1994) 37: 321-327] Key words IDDM, first-degree relatives, familial aggregation, recurrence risk, life-table analysis.The aetiology of IDDM is still rather poorly understood. The major genetic predisposition is conferred by HLA-class II genes [1-3] but genes outside this region may also confer susceptibility [4][5][6][7][8]. Furthermore, environmental factors are thought to be important for the initiation of the disease [9][10][11]. Epidemiological studies may be helpful in increasing our understanding of IDDM and are essential for generation of preventive and interventive strategies aimed at the eradication of the disease. When studying familial aggregation of Received: 14 July 1993 and in revised form: 13 October 1993Corresponding author: Dr. J.Nerup, Steno Diabetes Center, Niels Steensens Vej 2, DK-2820 Gentofte, DenmarkAbbreviations: IDDM, insulin-dependent diabetes mellitus; SE, standard error.IDDM the composition and representativeness of the study population are obviously important for allowing direct comparisons between results from different regions and centres.Several studies have reported the prevalence of IDDM patients having affected first-degree relatives within a range of 5 to 19 % [13][14][15][16][17][18][19][20][21][22][23][24]. However, many of these studies are not directly comparable, primarily due to methodological differences. Often, the study populations comprised only children or adolescent probands [13,15,18,19,21,24], were designed as incid...