[Rheumatology] Eosinophilic granulomatosis with polyangiitis (Churg-Straus) [EGPA] 진단과 진단 후 검사

In 1990, the ACR established six criteria for the classification of EGPA in a patient with documented vasculitis. The presence of four or more of these criteria had a sensitivity of 85 percent and a specificity of 99.7 percent for EGPA: ​ ● Asthma (a history of wheezing or the finding of diffuse high pitched wheezes on expiration) ● Greater than 10 percent eosinophils on the differential leukocyte count ● Mononeuropathy (including multiplex) or polyneuropathy ● Migratory or transient pulmonary opacities detected radiographically ● Paranasal sinus abnormality ● Biopsy containing a blood vessel showing the accumulation of eosinophils in extravascular areas 실제적으로 eosinophilic granulomatosis with polyangiitis (Churg-Straus) [EGPA] 진단은 호산구증가증 (≥1500 cells/microL), 천식, rhinosinusitis, 폐 또는 영향 받은 조직 (예, eosinophilic infiltration을 보이는 피부, 말초신경, 위장관)의 조직병리검사에 근거합니다. 아니면 조직병리검사가 가능하지 않을 때 감별진단으로 다른 질환들을 배제한 후에 위와 같은 임상 특징들과 말초 호산구증가증을 보이는 환자에서 추정진단이 이루어질 수 있습니다. 일단 EGPA로 진단이 되면 콩팥, 심장, 위장관, 말초신경에 대한 평가를 시행해야 하는데 좋지 않은 예후와 관련이 있기 때문입니다. Renal involvement ​ 1. Urinalysis (예, hematuria, proteinuria, red blood cell casts) 2. Blood urea nitrogen, serum creatinine 3. Antineutrophil cytoplasmic antibody (ANCA)는 EGPA로 인한 GN 환자에서 거의 항상 양성입니다. Initial cardiovascular testing ​ 1. N-terminal pro-brain natriuretic peptide (NT-proBNP) 2. Cardiac troponin 3. Electrocardiogram 4. Transthoracic echocardiogram Cardiovascular tests ​ We typically obtain serum levels of NT-proBNP and cardiac troponin, an electrocardiogram, and a transthoracic echocardiogram (looking for wall motion abnormalities, mural thrombi, and valvular thrombi) as part of the initial evaluation of a patient with a diagnosis of EGPA even in the absence of symptoms suggesting cardiac disease. The rationale for this routine testing is that cardiac involvement is the leading cause of mortality due to EGPA, and approximately 40 percent of asymptomatic patients with a normal ECG have evidence of cardiac involvement with EGPA on echocardiogram. We use this initial testing to guide further imaging and decisions about endomyocardial biopsy. Evaluation for gastrointestinal involvement ​ 1. Specific symptoms (예, upper abdominal pain, diarrhea, hematochezia) 2. (May) upper or lower endoscopy with biopsy 3. 복수가 있는 환자는 복수 샘플을 얻어야 합니다. Peripheral nerve involvement ​ 1. Focused Hx/PE 이후 필요하다면 nerve conduction studies와 electromyogram (NCS/EMG) 2. Biopsy는 EGPA로 인한 알려진 systemic vasculitis 환자에서 일반적으로 필요하지 않음 ​ ​ REF. UpToDate 2020.08.23