Vasculitis Historical Aspect

It is thought that Hippocrates first described vasculitis symptoms in the fifth century BC. However, it has only been in the past 160 years that vasculitis has been fully recognized as a distinct group of diseases. In 1801, Dr William Heberden, a London physician, described the first case of Henoch-Schönlein purpura.

Heberden wrote of a 5-year-old boy who seized with pains and swelling in various parts, ometimes in his belly with urine tinged with blood. It was in 1837 that Johann Schönlein and then in 1874 that Eduard Henoch reported additional cases

Prof. Dr Adolf Kussmaul

In 1866, Dr Adolf Kussmaul described the first case of Polyarteritis Nodosa and for almost 100 years, most forms of Vasculitis were thought to be variants of PAN. It was Dr Kussmaul who noted that the disease was associated with inflammation within the walls of the blood vessels and the signs and symptoms he described constituted a new disease. In 1890, the most common of the systemic vasculitides, Giant Cell (Temporal) Arteritis, was described by English surgeon Jonathan Hutchinson in the British Archives of Surgery. It was not until 42 years later that Bayard Horton and colleagues at the Mayo Clinic performed the first temporal artery biopsy.

Professor Mikito Takayasu

Takayasu’s Arteritis is also known as ‘Pulseless Disease’. In 1908, Mikito Takayasu described the first case of what would become known as Takayasu’s Arteritis at the 1908 Japanese Society of Ophthalmology.

Leo Buerger

Also, in 1908, Leo Buerger of Mount Sinai Hospital in New York evaluated a series of leg amputations. He recognized that what he saw was a new disease which he called ‘thromboangiitis obliterans’ Eventually, this was known as ‘Buerger’s Diseases’ Unfortunately, Buerger did not link the disease with the main risk factor of cigarette smoking. In 1931, Heinz Klinger reported, in his thesis, on two patients who had died from an overwhelming systemic disease. He believed that infection was the cause, and his thesis was entitled ‘Variants of Periarteritis Nodosa’.

Friedrich Wegener

It was not until 1936 that Dr Friedrich Wegener described three patients in their 30s who presented with innocuous symptoms but quickly progressed and the patients died from kidney failure within months. He recognized that this was a new disease and distinct from Polyarteritis Nodosa. The disease was named Wegener’s Granulomatosis (now renamed Granulomatosis with Polyangiitis).

Dr Hulusi Behçet

It was in 1937 that Turkish physician, Dr Hulusi Behçet (Professor of Dermatology) described three patients with what was to become known as Behçet’s Disease (sometimes referred to as the Silk Road Disease). He had first recognized the disease in 1924. However, in his Third Book of Endemic Diseases, Hippocrates described patients with oral and genital ulcers, sensitivity of the eyes to light and some loss of vision which are, of course, some of the symptoms of Behçet’s disease.

Dr Tomisaku Kawasaki

In 1939, an autopsy was conducted on a 5-year-old girl who was admitted with a sore throat, high fever and red rash over her body. Her tongue resembled a ripe strawberry. The disease was referred to as ‘infantile periarteritis nodosa’. Then, in 1961, Dr Tomisaku Kawasaki recognized these symptoms in one of his patients. He undertook an epidemiological study and identified 50 patients with a disorder called ‘mucocutaneous lymph node syndrome’, which by the mid 1970s became known as ‘Kawasaki’s Disease’. Until 1948, Microscopic Polyangiitis was considered to be a form of PAN. It was English physician Charles Davson and his colleagues who distinguished MPA from classical PAN, as their findings showed that MPA tended to involve the smallest blood vessels within the kidney.

Jacob Churg and Lottie Strauss identified another branch of the Periarteritis nodosa family in 1951. Thirteen patients with asthma, fever, elevated eosinophils and inflammation of the blood vessels were described. It was the striking number of eosinophils that Churg and Strauss recognized as distinguishing this condition from patients with PAN. Churg Strauss Syndrome was recently re-named as Eosinophilic Granulomatosis with Polyangiitis.

Polymyalgia Rheumatica was first described in 1957 and the name polymyalgia rheumatica was given to the disease by Stuart Barber. Prior to 1957, many patients were misdiagnosed as having rheumatism. This information only highlights the more common of the vasculitides. It has been compiled from internet articles, particularly ‘From Kussmaul to Kawasaki - Thumbnail sketches in the history of vasculitis’ [Parts 1 and 2 (2002) http://vasculitis.med.jhu.edu]