## 4 Case Reports
### Case 2  
A 31-year-old female patient received diagnosis of EGPA because of sinonasal disease, severe asthma, peripheral eosinophil, peripheral neuropathy, purpurura, p-ANCA, and anti-MPO positivity. Because of the remarkable peripheral eosinophil (Table 2), other hypereosinophilic syndromes [46] were considered in the differential before making a definitive diagnosis of EGPA. Specifically, hematologic, parasitic, and allergic diseases were ruled out by means of specific investigations. The gastrointestinal (GI) complaints (Table 2) were addressed by means of upper and lower endoscopy of the GI tract, which only showed a spastic colon; random biopsies carried out along the upper and lower GI tract did not show eosinophil infiltration consistent with eosinophilic gastroenteritis, colitis, or esophagitis [47]. The patient was initially started on prednisone 1 mg/kg per day and obtained acceptable asthma control as well as remission of purpurura, gastrointestinal complaints, and constitutional symptoms just after one month of therapy. An improvement in symptoms related to peripheral neuropathy was also observed. A 50% reduction in peripheral eosinophil (\(\sim\)4000/\(\upmu\)L) was recorded after 4 weeks of therapy, along with normalization of inflammatory markers. At the 3-month follow-up, with the patient on oral prednisone 20 mg/day, clinical manifestations were in remission and eosinophil was mild (\(\sim\)600/\(\upmu\)L). However, tapering of oral steroids could not be further accomplished without incipient rebound of the circulating eosinophil count (\(\sim\)1200/\(\upmu\)L) and new asthma  
\begin{table}
\begin{tabular}{c c c} \hline \hline
**Patients** & **Clinical Manifestations at Onset** & **Laboratory Investigations** \\ \hline \multirow{6}{*}{Case 1, 9, 52 years} & Asthma & Eosinophilia (\(\sim\)1800/\(\upmu\)L) \\  & Nasal polyps & Elevated IgE (\(\sim\)2000 UI/L) \\  & Hemoplysis & p-ANCA positivity \\  & & Pruetinuria (500 mg/24 h) & Anti-myleoperoxidase positivity \\  & & Constitutional symptoms (malaise, fatigue) & Elevated ESR and CRP \\ \hline \multirow{6}{*}{Case 2, 9, 31 years} & Asthma & Eosinophilia (8600/\(\upmu\)L) \\  & & Nasal polyps & FIP1L1/PDGFRa absent \\  & Gastrointestinal complaints (nausea, vomiting, diarrhea, abdominal ramping) & p-ANCA positivity \\  & Purpura & Anti-myleoperoxidase positivity \\  & Peripheral neuropathants (right hand and right leg) & Elevated ESR and CRP \\  & Constitutional symptoms (fewer, malaise, fatigue, substantial weight loss) & \\ \hline \hline \end{tabular}
\end{table}
Table 2: Characteristics of the patients administered IL-5-targeted therapies.  
exacerbations. Because of the exceedingly high count of peripheral blood eosinophils at diagnosis, benzlizumab (30 mg s.c. every 4 weeks for the first 3 injections, then 30 mg s.c. every 8 weeks, according to the schedule approved for eosinophil asthma [48]) was empirically chosen for its ability to efficaciously deplete these cells. One month later, asthma was in remission again and oral prednisone could be successfully tapered off in the subsequent weeks. Three months into benzlizumab treatment, the patient was still in complete remission in all aspects of EGPA, her peripheral eosinophils were undetectable (0 cells/uL) and oral and inhaled steroids had been completely suspended. Benralizumab is still ongoing as the only maintenance therapy, 30 mg s.c. every 8 weeks.