Vasculitis

Vasculitis means inflammation of blood vessel walls. It is not one disease but a family of rare conditions grouped by the size of the vessels involved. Giant cell arteritis and Takayasu arteritis affect large arteries. Polyarteritis nodosa and Kawasaki disease affect medium ones. The ANCA-associated forms, IgA vasculitis and others affect small vessels. Behcet's disease, which is relatively common in Türkiye, affects vessels of all sizes.

Inflamed vessels can narrow, become blocked or leak, so that organs are starved of blood. Severity ranges from a rash limited to the skin to life-threatening disease of the kidneys, lungs or nerves. Modern immunosuppressive treatment brings most forms into remission, though relapses are common and long-term monitoring is required.

Symptome

• Fever, night sweats, weight loss and exhaustion lasting weeks
• A rash of raised purple spots, usually on the lower legs
• Aching joints and muscles
• A new headache, tender scalp, jaw pain on chewing or loss of vision in people over 50, which suggests giant cell arteritis
• A blocked, crusting or bleeding nose, sinus pain and hearing loss
• Coughing blood or breathlessness
• Blood or protein in the urine, or swollen ankles
• Numbness, or a sudden foot drop or wrist drop
• Repeated mouth and genital ulcers with eye inflammation, which suggests Behcet's disease

Ursachen und Risikofaktoren

Most forms are autoimmune, with no single known trigger. The immune system damages vessel walls through autoantibodies such as ANCA, through deposits of immune complexes, or through direct attack by immune cells. Genetic background plays a part, for example HLA-B51 in Behcet's disease. Secondary vasculitis can be caused by infections, including hepatitis B and C and endocarditis, by medicines such as propylthiouracil and hydralazine, by cocaine contaminated with levamisole, by cancers, and by other autoimmune diseases such as lupus and rheumatoid arthritis. Giant cell arteritis occurs almost only after the age of 50. Kawasaki disease and IgA vasculitis mainly affect children.

Wie es diagnostiziert wird

• Clinical assessment: The pattern of organs involved points to the type. Conditions that mimic vasculitis, such as infection, clots and cholesterol emboli, have to be excluded.
• Blood and urine tests: Inflammatory markers, blood count, kidney function, and urine dipstick and microscopy, which is the quickest way to find kidney involvement.
• Immunology: ANCA with PR3 and MPO antibodies, ANA, complement levels, cryoglobulins and hepatitis serology.
• Biopsy: Of the skin, kidney, temporal artery, nerve or nasal lining. A biopsy gives the firmest diagnosis.
• Imaging: Ultrasound of the temporal arteries, CT or MR angiography, PET-CT for large-vessel disease, and chest CT.
• Organ-specific tests: Nerve conduction studies, echocardiography and eye examination as required.

Behandlungsmöglichkeiten

• Glucocorticoids: High-dose steroids control inflammation rapidly. For suspected giant cell arteritis with visual symptoms they are started the same day, before the biopsy.
• Rituximab or cyclophosphamide: Used to induce remission in ANCA-associated vasculitis with organ-threatening disease. Avacopan reduces the amount of steroid needed.
• Maintenance therapy: Rituximab, azathioprine, methotrexate or mycophenolate for 2 years or longer.
• Tocilizumab: For giant cell arteritis, to allow the steroid dose to be reduced.
• Treatment of Behcet's disease: Colchicine, azathioprine, apremilast or anti-TNF medicines, according to the organs involved.
• Plasma exchange and dialysis: For selected patients with severe kidney disease or bleeding in the lungs.
• Treating the underlying cause: Antiviral therapy for hepatitis-related forms, and stopping any drug responsible.
• Protection from the side effects of treatment: Bone protection, prevention of infection, vaccinations and cardiovascular risk control.

Wenn es dringend ist

Sudden loss of vision or double vision, coughing blood, severe breathlessness, a marked fall in urine output, severe abdominal pain, chest pain or sudden weakness of a limb need emergency care locally on the same day. Vision lost from giant cell arteritis does not return, so do not wait.

Reisen nach Türkiye zur Behandlung

Active vasculitis is not something to take on a journey. It needs prompt treatment and close monitoring of blood tests in the place where you live. Once you are stable, a visit can offer a second opinion on the type and the treatment plan, specialised imaging such as PET-CT or vascular ultrasound, a biopsy review, or the opinion of rheumatologists with wide experience of Behcet's disease. Infusions such as rituximab are repeated every 6 months and need screening for infection and follow-up, so they are best given at home.

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