Eight months ago he underwent a double lung transplant from which he was slowly recovering before kidney problems struck again.
"All at McLaren are deeply saddened to learn that our friend, colleague and 1984 Formula 1 World Champion, Niki Lauda, has passed away".
"His unique achievements as an athlete and entrepreneur are and will remain unforgettable", the family's statement said. He was the only driver to win championships for McLaren and Ferrari.
He underwent two kidney transplants, the second kidney donated in 2005 by his then-girlfriend Birgit Wetzinger, a former flight attendant for his airline whom he married in 2008.
The win came in an Alpina BMW 3.0 CSL together with Hans-Peter Joisten, this a period in his career when Lauda competed regularly in sports and touring vehicle machinery (pic above from that year's 6-hour race where the same duo topped their class and finished third overall).
Lauda suffered severe burns to his head and wrists, as well as having his lungs burnt from the toxic fumes. It was during this run in 1976 when Lauda survived the horrific crash at the Nurburgring that left him severely burnt.
The three world titles were won in 1975, 1977 and 1984.
But six weeks after receiving last rites in his hospital bed, Lauda surprised the world of motorsport by taking part in the Italian Grand Prix in Monza. In 1985 he went into full-time management of the airline after his 1985 retirement from F1 racing.
Along the way, Lauda never lost touch with F1, other than authoring a number of books, he was also a popular, if outspoken, pundit for RTL.
Facing tough competition from McLaren's James Hunt - their rivalry featured in the Ron Howard-directed movie Rush - Lauda appeared on course to defend his title in 1976 when he crashed at the Nuerburgring during the German Grand Prix.
He recalled "shaking with fear" as he changed into second gear on the first day of practice and thinking "I can't drive".
In his third season with the Woking outfit Lauda secured his third title, beating teammate Alain Prost by just half-a-point.
Appointed a consultant at Ferrari by Luca di Montezemolo, Lauda eventually sold his airline to Austrian Airlines, subsequently going on to form another airline, Niki, which eventually merged with Air Berlin.
Niki Lauda was considered a calculating racer and made his F1 debut in 1971. "Rest in peace Niki".
He partnered with budget carrier Ryanair on Niki's successor, LaudaMotion.
He was also Mercedes AMG Petronas F1 Team's non-executive chairman and was involved in the signing of Lewis Hamilton.
Team principal Toto Wolff said: "Niki will always remain one of the greatest legends of our sport - he combined heroism, humanity and honesty inside and outside the cockpit".
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