The vasculitides are defined by an inflammation of the blood vessels. The diseases are often classified based on the size of involved blood vessels and based on specific histological as well as immunological changes. The following diseases belong to the vasculitides: Granulomatosis with polyangiitis (formerly Morbus Wegener), eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), microscopic polyangiitis, giant cell arteritis, polymyalgia rheumatica, Takayasu arteritis, polyarteritis nodosa, Behcet`s syndrome and cryoglobulinemia. While all these diseases show inflammation of the blood vessel wall, important differences exists in the type and discipution of the involved blood vessels. Accordingly the clinical symptoms, involved organs, diagnostic tests and available therapeutic options are strongly depending on the individual vasculitis.
The symptoms of vasculitides depend on the involved blood vessels. Typical patterns of organ and blood vessel involvement exist for the specific vasculitides. For example, giant cell arteritis, polymyalgia rheumatica and Takayasu arteritis often involve large blood vessels. In giant cell arteritis, the main blood vessel of the eye is frequently involved which can lead to severe and sometimes irreversible visual loss.
Granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis belong to the ANCA-associated vasculitides and often involve smaller blood vessels. The upper and lower respiratory tract and the kidney, but also other organs like peripheral nerves and the skin are involved. Typical symptoms include among others breathing problems, symptoms of renal insufficiency, nerve pain and different forms of skin inflammation.
According to the heterogeneous organ involvements, the vasculitides often require very different diagnostic tests. In addition to imaging of the blood vessels using ultrasound, angiography and MRI-angiography additional imaging tests such as computer tomography of the lungs and the upper respiratory tract are often ordered. In many cases, the diagnosis of vasculitides requires a tissue biopsy and histological examinations. Laboratory analyses include inflammatory markers and measurement of autoantibodies. These assessments have to be tailored to the individual patient based on the suspected vasculitis and the stage and severity of the disease.
The vasculitides are chronic inflammatory diseases and therefor require immunosuppressive therapy in many patients to prevent severe irreversible organ damage. Initially, corticosteroids are frequently used, which are then gradually replaced by other corticoids sparing chronic immunosuppressive therapies. In recent years more specific anti-inflammatory treatments (biologics) have become available, which have improved outcome and tolerability of these treatments. In our clinics, these modern therapies are assessed and discussed with the patient, and an individualized treatment plan is developed.
Depending on the organ involvement, the vasculitides require a close collaboration with other divisions including, but not limited to dermatology, angiology, pulmonology, neurology, ophthalmology and clinical immunology.