Churg–Strauss syndrome

The Churg -Strauss syndrome ( CSS) is a granulomatous (eg: " granule -forming " ) inflammation of the small blood vessels in the affected tissue of certain inflammatory cells called eosinophils, infiltrated ( roughly: " wanders " ) is. It is for the most part proven associated with the increase in concentration of a specific sub-class of antibodies (ANCA (see below) ) and relates to smaller arteries. Due to the inflammatory response tissues and organs are damaged (see below). The name comes from the pathologists Jacob Churg and Lotte Strauss. Synonyms are allergic granulomatous angiitis and granulomatous small -vessel vasculitis.

Pathogenesis

The cause of the disease is largely unknown. An ANCA -associated autoimmune component is suspected. So the treatment is immunosuppressive. In patients diagnosed with cytokines interleukin -17A and interleukin -23 are elevated in serum.

Pathology

Histologically shows a strong increase of eosinophils in the tissue ( Gewebseosinophilie ), with involvement primarily of small blood vessels ( with destruction, blood clot formation and the resulting infarcts ). In addition, the inflammation can spread directly to various organs such as the heart, with the consequence of eosinophilic myocarditis.

Symptoms

The CSS has three different clinical stages:

The vasculitic phase usually begins about three years after the beginning of the first phase, but can be delayed even by decades. Furthermore, there occurs a contamination of lung, heart, kidney, skin and peripheral nervous system. The following symptoms are typical of the CSS:

In the laboratory, one can find a eosinophilia and anemia and signs of inflammation ( elevated ESR and CRP elevation ). Additionally you'll find antineutrophil cytoplasmic antibodies (p- ANCA ), increased IgE LSpiegel and an elevated rheumatoid factor.

In the radiograph of the chest is found in 25-75 percent of cases on both sides, unevenly distributed shadowing in the lungs.

Proving for the CSS is the biopsy of an affected organ or, if this is not present, the biopsy of muscle or nerve tissue ( then mostly from the sural nerve ).

CSS is like all vasculitides complex and difficult to diagnose. The general symptoms above do not all occur. In an unclear clinical picture but it is always useful to include a vasculitis as a trigger with.

Therapy

In most cases, treatment with prednisone is sufficient to control the symptoms, in a few cases such as cyclophosphamide chemotherapy are used. Other treatment options include the use of methotrexate, interferon - α, immunoglobulins, or plasmapheresis.

Nomenclature

The Churg -Strauss syndrome is one of the manifestations of systemic necrotizing inflammatory diseases of the small and medium-sized arteries. Had reported from classical periarteritis nodosa ( polyarteritis ) starting over which Kussmaul and Maier in 1866, Wegener 1939 described a granulomatous vasculitis of the upper respiratory tract as a separate syndrome. Churg and Strauss set in 1951 before another disease with bronchial asthma and peripheral eosinophilia. They called this variant originally " allergic granulomatosis " and " allergic angiitis ".