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.
Adolf Kussmaul, who lived and practiced medicine in the 19th century, is known for his clinical skills, his scientific acumen, his gift for teaching, and his mastery of diverse areas of knowledge. He was a contemporary of such luminaries as pathologist Rudolf Virchow. In the rheumatology community, he is best known for describing the first case of polyarteritis nodosa (PAN).
FIRST CASE
In the first volume of the first edition of German Archive for Clinical Medicine, Kussmaul, along with his pathology associate Rudolf Maier, reported the case of Carl Seufarth, a 27-year-old tailor’s journeyman. Seufarth arrived at the University of Freiburg internal medicine clinic on May 4, 1865, at 10 am. Kussmaul was at that time head of medicine at Freiburg. Seufarth’s journeyman’s log recorded that he had been healthy when he left his hometown of Gernsbach in southwest Germany on January 30, 1865. His entry indicated that he was 5 feet 2 inches tall, was of strong build, and had healthy facial color.
Kussmaul’s 1866 description of Seufarth upon his arrival at the clinic is among the most memorable passages in medical literature:
Despite his frail appearance, Seufarth was able to walk into the hospital and climb the two flights of stairs to the internal medicine clinic without assistance. He had had a cold followed by a productive cough in the autumn of 1864, but felt well afterward. In the 8 days prior to admission to the University of Freiburg, he developed diarrhea and frequent chills with fevers and sweats. He had felt unwell for the preceding 2 to 3 weeks, during which he was hospitalized briefly for scabies, wandered from one place to another, and eventually arrived in Freiburg. Freiburg police imprisoned him on May 2 for begging and brought him to the internal medicine department on May 4 because of weakness.
Over the next several days, Seufarth experienced rapidly developing weakness, numbness in the left hand and eventually other extremities, and paralysis of the arm and hand muscles. He was closely monitored at the clinic with his temperature recorded every morning and evening. On the 28th day of hospitalization, pea-sized nodules were discovered in the subcutaneous skin of the abdomen and chest. By June 2, the patient was in a state of extreme weakness. He died on June 3, 1865, at 2 am.
This description is what we understand today as typical of vascular involvement in PAN. Maier also examined the tissue microscopically. In his report, he described the aneurysmal dilatations, narrowings, and inflammation occurring at the branches of the arteries. His sketch of involved organs depicted neutrophilic infiltration into the walls of the vessels.
When consulted by Kussmaul for a second opinion, pathologist Rudolf Virchow said he had not observed patients with disease similar to that of Seufarth. In his archives, however, he later found a specimen of an aneurysm in a branch of the superior mesenteric artery.
Kussmaul and Maier published the case under the title “On a previously undescribed peculiar arterial disease (periarteritis nodosa) accompanied by Bright’s disease and rapidly progressive general muscle weakness.” “Periarteritis nodosa” was later termed “polyarteritis nodosa” to better describe the inflammation of multiple medium-and small-vessel arteries rather than inflammation around the arteries as Maier had initially envisioned it.
BIOGRAPHICAL NOTES
The son of a German army surgeon, Kussmaul was born in 1822 in Graben near Karlsruhe, a small town in the Black Forest of southwestern Germany. Kussmaul began his medical studies at the University of Heidelberg in 1840. That same year, he constructed the first ophthalmoscope. The device did not function as intended because he had not discovered the light orientation needed to prevent the iris from contracting. But, as he later said, “It was the best ophthalmoscope of the time. Its only drawback was that it did not work.”
After graduating from the University of Heidelberg, Kussmaul went into private practice in Wiesloch. He returned to the University a year later, after having developed pericarditis, where he served as an assistant in 1846 and 1847 and engaged not only in medicine and medical discovery, but also poetry, publishing, and social movements. He founded a magazine that published short stories, poetry, and spoofs on the government; and he coined the term “Biedermeier,” which refers to a furniture style as well as a German social movement.
With plans to further his medical education, Kussmaul and his friend, Edward Bronner, traveled to Vienna and Prague in 1847 and 1848. In Vienna, they met the anatomic pathologist Karl Rokitansky. Although the young men hoped to study with the renowned scientist, they were soon dissuaded by Rokitansky’s clear dislike of working with students. He also had little use for patients, holding that the best patient was a dead patient because of all that one could learn by doing an autopsy.
Kussmaul and Bronner returned to Germany, Kussmaul having been called to serve as a physician in the Baden battalion during the German-Danish war. There, he contributed significantly to the health of the army by insisting that wounded soldiers not be bled—a common treatment at that time that actually accelerated the deaths of many soldiers in the field.
