musculoskeletal and connective tissue disorders
Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Symptoms and Signs of EGPA
The syndrome has 3 phases, which may overlap: Prodromal: This phase may persist for years. Patients have allergic rhinitis, nasal polyposis, asthma, or a combination. 2nd phase: Peripheral blood and tissue eosinophilia is typical. Clinical presentation, which may resemble Löffler syndrome, includes chronic eosinophilic pneumonia and eosinophilic gastroenteritis. 3rd phase: Potentially life-threatening vasculitis develops. Organ dysfunction and systemic symptoms (eg, fever, malaise, weight loss, fatigue) are common in this phase. However, the phases do not necessarily follow one another consecutively, and the time interval between them varies greatly. Various organs and systems may be affected: Respiratory:Asthma, often with onset during adulthood, occurs in most patients and tends to be severe and corticosteroid-dependent. Sinusitis is common, but not destructive, without severe necrotizing inflammation. Patients may be short of breath. Transient patchy pulmonary infiltrates are common. Neurologic manifestations are very common. Multiple Mononeuropathy occurs in up to three fourths of patients. Central nervous system involvement is rare but can include hemiparesis, confusion, seizures, and coma, with or without cranial nerve palsies or evidence of cerebral infarction. Cutaneous:The skin is affected in about one half of patients. Nodules and papules appear on extensor surfaces of extremities. They are caused by extravascular palisading granulomatous lesions with central necrosis. Purpura or erythematous papules, due to leukocytoclastic vasculitis with or without prominent eosinophilic infiltration, may develop. Musculoskeletal:Arthralgias, myalgias, or even arthritis can occur. Cardiac:Cardiac involvement, a major cause of mortality, includes heart failure due to myocarditis and endomyocardial fibrosis, coronary artery vasculitis (possibly with myocardial infarction), valvular disorders, and pericarditis. The predominant histopathologic finding is eosinophilic myocarditis. Gastrointestinal:Up to one third of patients present with gastrointestinal symptoms (eg, abdominal pain, diarrhea, bleeding, acalculous cholecystitis) due to eosinophilic gastroenteritis or mesenteric ischemia due to vasculitis. Renal:The kidneys are affected less often than in other vasculitic disorders associated with antineutrophil cytoplasmic autoantibodies. Typically, pauci-immune (few if any immune complexes), focal segmental necrotizing glomerulonephritis with crescent formation is present; eosinophilic or granulomatous inflammation of the kidneys is rare. Renal, cardiac, or neurologic involvement indicates a worse prognosis. 