The syndrome Churg-Strauss (SCS), granulomatosis with polyangiitis eosinophilic also called angiitis allergic granulomatosis is a systemic vasculitis necrotizing that affects the vessels of small and medium caliber and which is associated with eosinophilic granulomas extravascular, peripheral eosinophilia and asthma.

It was first described by pathologists J. Churg and L. Strauss in a series of autopsies on patients with severe asthma. It is a rare syndrome of unknown etiology that affects both genders and all age groups. Patients with SCS generally respond well to therapy withglucocorticoids , although relapses are frequent after discontinuation. Early diagnosis and treatment generally lead to a good prognosis, with 90% survival one year after diagnosis. Differentiating between granulomatosis and polyangiitis can be difficult, however the increasing use of ANCA ( anti-neutrophil cytoplasmic antibody ) dosage allows for a distinction, since in that one there is the presence of the c-ANCA (cytoplasmic) pattern and in that of p- ANCA (perinuclear).

The etiology of SCS is unknown. The proposed hypotheses include an autoimmune process, possibly associated with genetic and environmental factors.

The antagonists of leukotriene receptors ( ARL ) , including zafirlukast and montelukast were associated with the SCS development in the last decade. This relates likely effect with the suspension of corticosteroids after the start of intake of ARL s in patients with SCS hitherto attenuated or prescription ARLs patients with asthma phase that was phase – SCS reality unidentified. In fact, there are case reports of CSS after the initiation of inhaled corticosteroids andomalizumab and a recent crossover study showed that several of the asthma control medications are associated with the onset of SCS, supporting the hypothesis that what is associated with the onset of SCS is a worsening of asthma and not a specific drug.

The pathophysiology of CSS is still unclear and poorly defined. It presents a particular feature is the prominent elevation of blood and tissue eosinophilia, which is related to the activity and severity of the disease. In patients with active pathology, eosinophils express activation surface markers, and secrete proteins that lead to tissue damage. The interleukins does not have a well – defined role in the SCS. But it seems to be maintained by the increased release of IL-5 , produced mainly by activated Th2 lymphocytes. Indeed, the anti-IL5 antibody mepolizumab was shown to be effective in refractory SCS in a recent study. Although SCS is mainly associated with a Th2 profile, recent studies have also demonstrated possible imbalances in the activity of Th1, T regulatory and Th17 lymphocytes. Anti-neutrophil cytoplasmic antibodies ( ANCA ), specificallyanti- myeloperoxidase , appear to have a pathogenic role in vasculitis lesions, possibly through the activation of neutrophils, leading to the release of reactive oxygen species and proteolytic enzymes. Proposed roles for eosinophils and ANCA in pathogenesis of the disease are in agreement with recent descriptions of different manifestations of the disease in patients with and without ANCA.

The clinical manifestations of CSS are generally described in three phases:

The first phase ( prodromal ) can last for several years. The predominant features are atopic asthma and rhinitis, which is generally difficult to control.
In the second phase ( eosinophilic ), there is an intense tissue infiltration by eosinophils in several organs, including the lungs and the gastrointestinal (GI) tract, eventually progressing to Loeffler syndrome or eosinophilic gastroenteritis.
The third stage (vasculitic) is characterized by a marked systemic vasculitis, in which general symptoms usually precede the involvement of the various organs by this process.
One of the systems that is frequently affected by vasculitis is the peripheral nervous system , with involvement of the vasa vasorum and development of multiplex mononeuritis. The GI tract is also affected, with manifestations of abdominal pain, gastroenteritis or even an acute abdomen. Cardiac involvement can present as myocarditis , heart failure , pericarditis or even coronary vasculitis and consequent myocardial ischemia. About half of SCS-related deaths are caused by heart damage.

This video I do not own. I put here just for learning purposes.

The respiratory system is involved in all patients to varying degrees, not only with asthma in the first clinical phase, but also with infiltrates on chest X-rays, which are present in 37 to 77% of all patients. The pleural effusion can also be observed in up to one third of patients.

Clinically significant kidney disease is less frequent and less severe than in other forms of vasculitis.

Please learn more in these websites:

Translate »
Skip to content