PDF | Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis of the small Resumo A síndrome de Churg-Strauss (SCS) é uma vasculite sistémica . Introdução: O S. de Churg-Strauss (SCS) é uma entidade clínica em que um quadro de asma e eosinofilia coexiste com um atingimento multissistémico. A síndrome de Churg-Strauss (SCS) é uma vasculite sistémica necrotizante, que afeta os vasos de pequeno e médio calibre e se associa a granulomas.
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Resumo A síndrome de Churg-Strauss (SCS) é uma vasculite sistémica Abstract Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis of the . Epub Dec Churg-Strauss syndrome. Greco A(1), Rizzo MI(2), De Virgilio A(3), Gallo A(4), Fusconi M(1), Ruoppolo G(1), Altissimi G(5), De Churg- Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with. Churg–Strauss syndrome (CSS) is a small and medium vessel vasculitis characterized and vasculitic phases) although they do not always occur successively.
Case series with a focus on neurologic involvement are not common. Methods: In this monocentric study, consecutive patients of our hospital with first diagnosis of CSS based on the criteria of the American College of Rheumatology were included between and Data were obtained prospectively. Results: Fourteen patients were included. All patients had a hypereosinophilia and a history of asthma. Twelve of 14 patients had a neurologic involvement, mainly as an acute or subacute multiplex mononeuropathy eight patients or an axonal polyneuropathy three patients.
Patient 3 was a year-old man with personal history of asthma and rhinitis.
He showed symptoms of mixed axonal polyneuropathy over a 2-month period with eosinophilia, positive ANCA test, and interstitial pneumonitis. The patient started induction treatment with decreasing doses of corticosteroids and 5 doses of cyclophosphamide. Clinical response was excellent.
After that, he was treated with azathioprine as maintenance treatment. He is currently asymptomatic. The incidence of CSS is 2. The syndrome is more frequent in men.
It can appear in patients with a history of allergy and atopy, and it may develop over 30 years before any systemic manifestations are observed. It manifests in 3 stages and asthma seems to present years before the other symptoms do. Involvement mainly manifests as mononeuritis multiplex or distal symmetric sensorimotor axonal polyneuropathy.
The dose is then progressively decreased to continue with maintenance treatment. Figure 3. Hematoxylin and eosin cardiac biopsy specimen at presentation showing eosinophilic infiltration A. Myocardial biopsy, after glucocorticoid and immunosuppressive therapy, showing complete resolution of eosinophilic myocarditis B.
Churg—Strauss syndrome was then diagnosed as four out of six criteria were present in this patient: 1 asthma; 2 eosinophilia; 3 mononeuropathy; 4 extravascular eosinophils. On day one of treatment, the patient's symptoms improved significantly and levels of blood eosinophils fell to 0.
Echocardiography performed after 18 days of treatment showed resolution of the pericardial effusion as well as of the left ventricular wall motion abnormalities. Control coronary angiography one year after initiation of therapy showed complete regression of coronary stenotic lesions Figure 2 C and D and a myocardial biopsy confirmed resolution of eosinophilic myocarditis Figure 3 B.
The patient was stable and symptom-free at one year of follow-up under chronic therapy. Discussion We describe the case of a patient with newly diagnosed CSS presenting with extensive myocarditis, which is an unusual clinical manifestation of this disease.
This case report highlights the possibility of cardiac involvement in patients with CSS and calls attention to this differential diagnosis in the evaluation of myocarditis.
This diagnosis is mostly overlooked because major cardiac problems are rarely the presenting manifestations of vasculitis. Involvement of the heart has been described in the third stage of the disease, as observed in our patient.
It is usually associated with vasculitic lesions in the myocardium and coronary vessels causing peri myocarditis, heart failure, cardiac tamponade, myocardial infarction, or pericardial effusion. The myocardial damage is caused by vasculitis leading to coronary arteritis and coronary occlusion, through the release by activated eosinophils of toxic mediators causing direct myocardial damage, 12 or by replacement of the myocardium with granulomas and scar tissue.
Patients with acute multiorgan disease should receive intravenous glucocorticoid eg. The higher dose is used for patients with more aggressive disease, including those with cardiac involvement.
Clinical remission of isolated pericarditis, without other visceral involvement, can be obtained with corticosteroid therapy alone. Prevalence of serious eosinophilia and incidence of Churg-Strauss syndrome in a cohort of asthma patients.
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Clinical study and long-term follow-up of 96 patients.
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