History of vasculitis: The life and work of Adolf Kussmaul
ABSTRACTAdolf Kussmaul is well known for his contributions to the science of medicine and the specialty of rheumatology. A much-loved teacher and respected physician and researcher, Kussmaul’s desire to understand disease, his careful clinical observations, and his innovative thinking in medical technology mark him as a pioneer in modern rheumatology.
ACADEMICIAN, SCIENTIST, AND CLINICIAN
Shortly after his 1850 marriage to Luise Amanda Wolf, the daughter of a famous surgeon, Kussmaul developed an ascending polyradiculopathy, which at one time was called Landry-Kussmaul paralysis and later Guillain-Barré syndrome. This condition, along with his previous history of pericarditis, stimulated Kussmaul’s pursuit of medical knowledge for better understanding of his own afflictions as well as medicine in general.
He completed his doctoral dissertation at the University of Würzburg in 1853. There, he worked with pathology professor Virchow, who is known as the father of the theory of coagulation and the cellular theory of disease. It is perhaps less well known that in a treatise on histopathology in 1847, Virchow proposed that vasculitis actually might occur in blood vessels and originate in the adventitia. This profound insight was lost at the time because of inadequate understanding of vasculitic disorders.
Returning to the University of Heidelberg in 1854, Kussmaul earned the rank of assistant professor of medicine and, by 1857, professor of medicine. Two years later, he relocated to the University of Erlangen as a professor of medicine. His inaugural lecture at the University of Erlangen was the presentation of two cases of Landry-Kussmaul paralysis. Kussmaul’s research at Erlangen focused on differentiating the symptoms of mercurialism from syphilis (mercury was used for the treatment of syphilis).
Kussmaul was then called to the University of Freiburg in 1863 as head of the department of medicine. Among Kussmaul’s achievements at the University of Freiburg in the 1860s were the description of paradoxical pulse in obstructive pericarditis that we know as the Kussmaul pulse, and the description of the breathing characteristic of diabetic acidotic coma that we know as Kussmaul respiration. There he also performed the first gastroscopy on a sword-swallowing circus performer using a derivation of a laryngoscope; unfortunately, again his invention was thwarted by lack of an adequate light source. He also studied peptic ulcer disease and described a technique for dilating a stenosed peptic ulcer lesion with a balloon device. He later worked with Czerny and Billroth to develop the surgical procedure used routinely for nearly 100 years to relieve peptic ulcer disease prior to the introduction of drugs such as ranitidine.
RHEUMATOLOGY “WORMS”
Kussmaul and Maier initially published the Seufarth case in abstract form and called it “human worm aneurysm,” because they thought that the vascular pea-shaped or pea-sized structures represented worm and nematode infiltration. When they examined the specimens microscopically, however, they realized that they were viewing an inflammatory disease process.
Ironically, vessel disease of the PAN type was described in 1852 by Rokitansky. Rokitansky reported finding mesenteric aneurysms in the branch points of the arteries; however, because he eschewed technology, he did not examine the specimen microscopically and failed to recognize the inflammatory process. His student, Hans Eppinger, revisited the specimen some 30 years later and, under microscopic examination, clearly defined the aneurysmal dilatations and inflammatory infiltrates (Figure 2).
A final rheumatology worm episode occurred late in Kussmaul’s career in Strasburg, where he had become head of the department of medicine in 1878. Kussmaul asked his assistant and biographer, Albert Kahn, to administer naphthalene to a patient to eradicate intestinal worms. Strangely, the worms survived, but the fever resolved. Due to a pharmacy error, acetanilide, an anti-inflammatory marketed by Bayer, had been dispensed rather than naphthalene. Bayer subsequently marketed the product as Antifebrin.
REMEMBERED AND COMMEMORATED
Kussmaul was a much-loved teacher and a well-respected physician. After he retired in 1888, he returned to Heidelberg as emeritus professor of medicine. He died in 1902 at age 80. His desire to understand disease, his clinical observations, his teaching abilities, and his ability to apply medical technology to the bedside all played roles in his contributions to clinical medicine. One of several Kussmaul commemoration sites is a lunette in Lenox Hill Hospital, New York, New York, where his portrait plaque is displayed alongside those of Ismar Boas and Carl Anton Ewald, the founders of modern gastroenterology.
