Vasculitides

Vasculitides are inflammatory rheumatic diseases characterized by a tendency to inflammation of (mostly) arterial blood vessels. Vasculitis is the immune-reactively triggered inflammation of blood vessels. The following forms of vasculitis can be distinguished:

  • Primary forms of vasculitis – without an identifiable cause.
    • Anti-GBM (glomerular basement membrane) disease (synonym: Goodpasture’s syndrome; ICD-10 M31.0) – glomerular (affecting the kidney) and pulmonary (affecting the lungs) capillaries are affected, or in other words, it is a combination of glomerulonephritis (inflammation of the glomeruli (renal corpuscles) of the kidneys) with pulmonary hemorrhage
    • Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome (CCS) (synonyms: allergic granulomatous angiitis; Churg-Strauss granulomatosis; Churg-Strauss syndrome; CSS; ICD-10 M30. 1) – granulomatous (roughly: “granule-forming”) inflammation of small to medium-sized blood vessels in which the affected tissue is infiltrated (“walked through”) by eosinophilic granulocytes (inflammatory cells) [see below “Eosinophilic granulomatosis with polyangiitis (EGPA)”].
    • Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis (synonyms: Allergic Angiitis and Granulomatosis; Glomerular Disease in Wegener’s Granulomatosis; Glomerular Disorder in Wegener’s Granulomatosis; Glomerulonephritis in Wegener’s Granulomatosis; Granuloma gangraenescens; granulomatous polyangiitis; granulomatosis Wegener; Klinger-Wegener-Churg syndrome; pulmonary granulomatosis; McBride-Stewart syndrome [granuloma gangraenescens]; Wegener’s disease; necrotizing respiratory granulomatosis; rhinogenic granulomatosis; giant cell granuloarteritis; giant cell granuloarteritis Wegener-Klinger-Churg; Wegener granulomatosis; Wegener-Klinger-Churg syndrome; Wegener-Klinger-Churg syndrome with pulmonary involvement; Wegener disease; Wegener disease with pulmonary involvement; Wegener’s granulomatosis (or in other order Wegener-Klinger-Churg syndrome); Wegener syndrome; ICD 10 M31. 3) – necrotizing (tissue dying) vasculitis (vascular inflammation) of the small to medium-sized vessels (small-vessel vasculitides), which is associated with granuloma formation (nodule formation) in the upper respiratory tract (nose, sinuses, middle ear, oropharynx) as well as the lower respiratory tract (lungs) [see below “Granulomatosis with polyangiitis“].
    • Isolated leukocytoclastic cutaneous vasculitis (ICD-10 L95.9) – by definition, is confined to the skin; occurs predominantly in association with infections or other precipitating events (e.g., drug ingestion)
    • Kawasaki syndrome (synonym: mucocutaneous lymph node syndrome, MCLS; M30.3) – acute, febrile, systemic disease characterized by necrotizing vasculitis of small and medium-sized arteries
    • Microscopic polyangiitis (MPA) (synonyms: mPAN; ICD-10 M31.7) – necrotizing (tissue dying) vasculitis (vascular inflammation) of the small (“microscopic”) blood vessels, although larger vessels may also be affected; it is similar to granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis associated with ANCA autoantibodies [see below “Microscopic polyangiitis“].
    • Polyarteritis nodosa (PAN, synonyms: Kussmaul-Maier disease, panarteritis nodosa; M30.0) – is a necrotizing vasculitis of the middle arteries that belongs to the group of autoimmune diseases; PAN can be triggered by viral infections (e.g., hepatitis B virus (HBV), hepatitis C virus (HCV) infection)
    • Schönlein-Henoch purpura [new: IgA vasculitis (IgAV)] (synonyms: Anaphylactoid purpura; Acute infantile hemorrhagic edema; Schönlein-Henoch disease; Purpura anaphylactoides; Purpura anaphylactoides; Purpura Schönlein-Henoch (PSH); Seidlmayer cocard purpura; Schönlein-Henoch purpura; Vasculitis allergica; D69. 0) – immunologically mediated vasculitis of the capillaries and pre-capillary and post-capillary vessels, usually without complications; as a multisystem disease, it preferentially affects the skin, joints, intestines, and kidneys [see “Schönlein-Henoch purpura” below].
    • Giant cell arteritis with arteritis temporalis and Takayasu arteritis (M31.5) – vasculitis of large and medium-sized arteries.
  • Secondary forms of vasculitis – occur in the setting of another disease, viz.

Sex ratio:In anti-GBM (glomerular basement membrane) disease, men are affected about twice as often as women.In eosinophilic granulomatosis with polyangiitis, women are affected about twice as often as men.Men and women are affected equally in granulomatosis with polyangiitis and microscopic polyangiitis. In polyarteiitis nodosa, men are affected approximately three times as often as women. In giant cell arteritis, women are affected twice as often as men. Frequency peak: The frequency of vasculitis in general increases with age. In northern countries, vasculitides occur more frequently than in southern countries.The frequency peak of anti-GBM (glomerular basement membrane) disease is between the ages of 20 and 40. The frequency peak of eosinophilic granulomatosis with polyangiitis is between 40 and 50 years of age.Granulomatosis with polyangiitis can occur at any age. The peak incidence of Kawasaki syndrome is almost exclusively in childhood (80%). The peak incidence of Schönlein-Henoch purpura is almost exclusively in childhood. The peak incidence of giant cell arteritis is beyond the age of 60 years. The prevalence of vasculitis is approximately 200 cases per 1,000,000 persons. The prevalence of granulomatosis with polyangiitis is approximately 5 cases per 100,000 persons. The prevalence of polyarteritis nodosa is less than 5 cases per 100,00 inhabitants. The incidence (frequency of new cases) of anti-GBM (glomerular basement membrane) disease is approximately 0.5-1 disease per 1,000,000 population per year. The incidence of eosinophilic granulomatosis with polyangiitis is approximately1-2 diseases per 1,000,000 population per year.The incidence of granulomatosis with polyangiitis is approximately 0.9 diseases per 100,000 population per year.The incidence of isolated leukocytoclastic skin vasculitis is approximately 15 diseases per 1,000,000 population per year. The incidence of microscopic polyangiitis is approximately 4 disease per 1,000,000 population per year. The incidence of Schönlein-Henoch purpura is approximately 15-25 diseases per 100,000 population per year. The incidence of giant cell arteritis (RZA) is approximately 15-20 diseases per 100,000 population per year in Northern Europe. Course and prognosis: Anti-GBM (glomerular basement membrane) disease is rapidly progressive (progressive), so early diagnosis is of great importance for the course. The disease rarely relapses, that is, rarely recurs. Isolated leukocytoclastic skin vasculitis usually heals without sequelae.The use of immunosuppressive therapy for ANCA-associated vasculitides (AAV) – granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis – has significantly improved the life expectancy of those affected in recent years.Recurrences are common, so patients should be monitored closely. Risk factors for recurrence include PR3-ANCA leading to a doubling of the recurrence rate, early cessation of glucocorticoid therapy, and lower total cyclophosphamide dose/duration of therapy. Infections can trigger relapses (recurrence of disease). The lethality (mortality relative to the total number of people with the disease) of Kawasaki syndrome is approximately 1%. The 5-year survival rate for eosinophilic granulomatosis with polyangiitis is over 80% with optimal therapy. The most common causes of death are myocardial infarction (heart attack) and heart failure.In granulomatosis with polyangiitis, the 5-year survival rate without adequate therapy is a few months (< 6 months). With adequate therapy, it is >85%.