Churg-straus disease
(Allergic Angiitis and Granulomatosis)
Churg-Straus disease is a systemic necrotizing vasculitis with granulomas and often affects the lungs.
Symptoms may be similar to those of classic polyarteritis nodosa plus pulmonary symptoms.
Diagnosis is by biopsy.
Treatment is similar to that of polyarteritis nodosa.
Churg-Straus disease (CSD) differs from polyarteritis nodosa in that it frequently involves the lungs ,may involve small and large vessels, cause granulomas and is strongly associated with asthma and eosinophilia.
The cause is unknown but CSD has been precipitated by lowering the dose of corticosteroids in patients taking leukotriene receptor antagonistis for asthma.
However ,eosinophilia and association with asthma suggests that hypersensitivity is involved in its pathophysiology.
CSD is uncommon with about 3 cases per million populations.
Mean age at on set is 44 years.
In CSD, granulomas form in vessels and tissues .Eosinophils accumulate in tissues and the blood, organs and organ systems commonly affected include the lungs,akin,cardiovascular system (e.g coronary artery vasculitis,hypertension), kidneys, peripheral nervous system and GI track.
Symptoms,Signs and Diagnosis
Clinical manifestations are similar to those of polyarteritis nodosa except that lung involvement is often the predominant abnormality. Wheezing is common,dyspnea and cough may occur and chest x-rays show pulmonary infiltrates.
The diagnosis should be suspected in patients who have wheezing or pulmonary infiltrates and symptoms or signs suggesting vasculitis in other organ systems . CBC with differential and Antineutrophil cytoplasmic antibodies (ANCA) should be obtained.
More than 80 % of patients with CSD have eosophil >=1000 cells/microL.
Some patients have a positive p-ANCA (ANCA that is reactive mainly to myeloperoxidase)
ANCA testing can help to differentiate CSD from Wegeners granulomatosis,as c-ANCA ( ANCA that is reactive mainly to proteinase-3) s typically negative in CSD.
The diagnosis of vasculitis is made by biopsy .prognosis and treatment are similar to those of polyarteritis nodosa, although CSD is generally more responsive to corticosteroids.
Churg-Straus disease is a systemic necrotizing vasculitis with granulomas and often affects the lungs.
Symptoms may be similar to those of classic polyarteritis nodosa plus pulmonary symptoms.
Diagnosis is by biopsy.
Treatment is similar to that of polyarteritis nodosa.
Churg-Straus disease (CSD) differs from polyarteritis nodosa in that it frequently involves the lungs ,may involve small and large vessels, cause granulomas and is strongly associated with asthma and eosinophilia.
The cause is unknown but CSD has been precipitated by lowering the dose of corticosteroids in patients taking leukotriene receptor antagonistis for asthma.
However ,eosinophilia and association with asthma suggests that hypersensitivity is involved in its pathophysiology.
CSD is uncommon with about 3 cases per million populations.
Mean age at on set is 44 years.
In CSD, granulomas form in vessels and tissues .Eosinophils accumulate in tissues and the blood, organs and organ systems commonly affected include the lungs,akin,cardiovascular system (e.g coronary artery vasculitis,hypertension), kidneys, peripheral nervous system and GI track.
Symptoms,Signs and Diagnosis
Clinical manifestations are similar to those of polyarteritis nodosa except that lung involvement is often the predominant abnormality. Wheezing is common,dyspnea and cough may occur and chest x-rays show pulmonary infiltrates.
The diagnosis should be suspected in patients who have wheezing or pulmonary infiltrates and symptoms or signs suggesting vasculitis in other organ systems . CBC with differential and Antineutrophil cytoplasmic antibodies (ANCA) should be obtained.
More than 80 % of patients with CSD have eosophil >=1000 cells/microL.
Some patients have a positive p-ANCA (ANCA that is reactive mainly to myeloperoxidase)
ANCA testing can help to differentiate CSD from Wegeners granulomatosis,as c-ANCA ( ANCA that is reactive mainly to proteinase-3) s typically negative in CSD.
The diagnosis of vasculitis is made by biopsy .prognosis and treatment are similar to those of polyarteritis nodosa, although CSD is generally more responsive to corticosteroids.
0 comments :
Post a Comment