Eosinophilic granulomatosis with polyangiitis

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Classification according to ICD-10
M30.1 Panarteritis with lung involvement.
Allergic granulomatosis.
Churg-Strauss granulomatosis
ICD-10 online (WHO version 2019)

The eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a very rare granulomatous (eg: "granule-forming"), inflammation of blood vessels , in which the affected tissue by certain inflammatory cells, the eosinophils , infiltrated (approximately: "wandered through"). For the most part, it has been shown to be linked to an increase in the concentration of a certain subclass of antibodies ( ANCA (see below)) and mainly affects small and medium-sized arteries of the lungs and other organs. Tissue and organs are damaged by the inflammatory reaction (see below). The name comes from the pathologists Jacob Churg and Lotte Strauss , who first described the disease in 1951.

Pathogenesis

The cause of the disease is largely unknown. An ANCA -associated autoimmune component is suspected. The treatment is also immunosuppressive . The cytokines interleukin-17 A and interleukin-23 in the serum are increased in sick people .

Prevalence

About 1–3 cases / 1 million people occur each year. The average age of the initial diagnosis is 48 years. More women than men are affected.

pathology

Pronounced tissue eosinophilia (red nucleated cells outside the vessels) with vascular damage, HE staining .
Greater magnification.

In fine tissue, there is a strong increase in eosinophilic granulocytes in the tissue (tissue eosinophilia ), with infestation especially of the small blood vessels (with destruction, blood clot formation and the resulting infarcts ). In addition, the inflammation can also spread directly to various organs, e.g. B. on the heart with the consequence of eosinophilic myocarditis .

Symptoms

The EGPA has three different clinical phases:

  1. allergic rhinitis and bronchial asthma
  2. eosinophilic inflammation of the lungs and digestive tract
  3. systemic blood vessel inflammation (vasculitis) with granulomatous inflammation

The vasculitic phase usually begins about three years after the beginning of the first phase, but it can also occur with a delay of decades. The lungs, heart, kidneys, skin and peripheral nervous system are also affected. The following symptoms are typical for EGPA:

  1. General symptoms (tiredness, flu-like symptoms such as fever and muscle pain, general malaise, weight loss in 70 percent of cases)
  2. Asthma (97 percent of cases)
  3. Sinus infection (61 percent of cases)
  4. allergic rhinitis
  5. Joint pain
  6. Skin manifestations ( purpura , skin nodules, hives )
  7. Heart symptoms that suggest a heart attack and myocarditis .
  8. Nerve inflammation
  9. through kidney involvement, high blood pressure and kidney failure
  10. Symptoms relating to the gastrointestinal tract (bleeding, "appendicitis" , inflammation of the pancreas , gastrointestinal complaints )

In the laboratory one finds eosinophilia and anemia as well as signs of inflammation (increased sedimentation rate and increased CRP ). Antineutrophilic cytoplasmic antibodies ( p-ANCA ), increased IgE levels and an increased rheumatoid factor are also found .

Chest x-rays show unevenly distributed shadows on both sides of the lungs in 25–75 percent of cases.

Evidence for EGPA is the biopsy from an affected organ or, if this is not available, the biopsy from muscle or nerve tissue (then usually from the sural nerve ).

Like all vasculitis, EGPA is complex and difficult to diagnose. The general symptoms given above need not all appear. If the clinical picture is unclear, however, it always makes sense to include vasculitis as a trigger.

Differential diagnosis

The hypereosinophilia syndrome and the clinically similar granulomatosis with polyangiitis must be distinguished .

therapy

If left untreated, the 5-year survival is only given as about 25%. One study reported a 72% chance of survival in 6 1/2 years for patients treated. The most common cause of death are cardiovascular diseases.

In many cases, prednisone therapy is sufficient to control symptoms. In the case of serious organ involvement, a combination with chemotherapeutic agents such as cyclophosphamide is the agent of choice. Other treatment options are the use of methotrexate , interferon- α, immunoglobulins or plasmapheresis . On December 12, 2017, the anti- interleukin -5 antibody mepolizumab received FDA approval for the treatment of adult patients with EGPA.

nomenclature

EGPA belongs to the group of systemic, necrotizing inflammatory diseases of the small and medium-sized arteries. Starting from the classic periarteritis nodosa (panarteritis), which Kussmaul and Maier reported in 1866, Wegener described granulomatous vasculitis of the upper respiratory tract as a separate syndrome in 1939. In 1951, Churg and Strauss presented another clinical picture with bronchial asthma and peripheral eosinophilia. They originally called this variant "allergic granulomatosis " and "allergic angiitis ".

literature

  1. ↑ E. Nogueira, S. Hamour, D. Sawant et al .: Serum IL-17 and IL-23 Levels and autoantigen-specific Th17 cells are elevated in patients with ANCA-associated vasculitis. In: Nephrol Dial Transplant . 2010; 25, pp. 2209-2217. PMID 20100727
  2. ^ A b c Kasper, Fauci: Harrison's Principles of Internal Medicine. 19th edition. tape 2 . McGraw-Hi11 Education, New York 2015, ISBN 978-0-07-180215-4 , pp. 2186-2187 .
  3. ↑ https://arznei-news.de/mepolizumab/

Initial description

  • J. Churg, L. Strauss: Allergic Granulomatosis, Allergic Angiitis, and Periarteritis nodosa. In: Am J Pathol . 27 (1951), pp. 277-301. PMID 14819261 (English)

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