Table 2 Studies and case series of monoclonal antibodies for the management of ICEP

Brenard et al. 2020 [19].

Multicenter, open-label, retrospective study (real-life study)

Median follow-up 9 months from diagnosis to start of mepolizumab. Median follow-up after initiation of mepolizumab 9 months (6–12 m)

12 patients, 2 excluded.

Total 10 (5 men, 5 women)

Mepolizumab 100 mg every 4 weeks (6 patients: approved dose for severe asthma; 4 patients: 300 mg every 4 weeks/EGPA and hypereosinophilic syndrome)

- Annual relapse rate

- Use of systemic corticosteroids before and after the start of mepolizumab

- Lung lesions, evaluated by CT before starting the biological and at the last follow-up while on the biological

-Patients with eosinophilia related to JAK1 inhibitors and PDGFRα- FIP1L1.

-Multiorgan involvement (heart, skin)

-Infections

-Findings secondary to other medications

Peripheral eosinophilia, BAL, lung function, CT, Comorbidities, and exposures

Relapse: symptoms (cough, dyspnea) with increased pulmonary eosinophils, radiographic or CT changes in the absence of infection.

-All had at least 1 relapse before mepolizumab.

No significant differences between doses of mepolizumab.

Annual relapse rate reduction.

Decrease in eosinophils at 3 months.

7 of 8 evaluated by CT had complete resolution, after the 6th month of mepolizumab. Two patients were evaluated with radiography at 6 months and no alterations were found.

OS dose reduction in 9 patients at the 3rd month. At the 6th month only one patient still took OS, but in low doses due to tolerance of the decline.

No patient had secondary events in the study.

Askin & Morris 2021 [20].

Retrospective Case Series

Not described

53 patients, 12 received biological.

Mepolizumab

Benralizumab

They do not describe doses, nor do they describe treatment allocation

Improvement in radiological, clinical, psychological changes; relapses; decrease or suspension of corticosteroid

Hypereosinophilic syndrome, patients who did not have a BAL study, or a report of eosinophils in BAL < 20%

Not described

All had sustained improvement in radiological and clinical changes, including lung function and psychological field.

There were no relapses.

OS decreased in all patients.

No patient had serious secondary events.

Tashiro et al. 2022 [21].

Retrospective Case Series

Patient 1:8 months Patient 2:4 months Patient 3:19 months Patient 4:17 months

30 patients with the diagnosis, 12 relapses (6 had > 2 relapses), 4 received anti-IL5 biologic

2 men aged 68 and 74: Mepolizumab 100 mg every 4 weeks. 2 women aged 67 and 37: Benralizumab 30 mg every 4 weeks × 3 doses, then 30 mg every 8 weeks

Relapses, decrease in eosinophils, withdrawal, or reduction of corticosteroid dose

Findings due to drugs, mycoses, parasitic diseases, EGPA, ABPA, and other systemic diseases.

Patients with T₀ > 37.5 °C, cough > 2 weeks, pulmonary infiltrates on CT, eosinophilia > 1000, BAL eosinophils > 25%, improvement of changes after initiation of steroids

1. Patient with type 2 diabetes, without relapses after starting benralizumab. Improvement at 4 weeks in FEV1 and absence of eosinophils. Systemic corticosteroid was withdrawn.

2. Patient without comorbidities, without relapses after starting benralizumab. Absence of eosinophils, there is no complete withdrawal of systemic corticosteroid, but dose reduction continues.

3. Patient with hypertension, hyperlipidemia, and heart failure, without relapses after the start of mepolizumab, at 4 weeks eosinophil reduction. Systemic corticosteroid was withdrawn.

4. Patient with type 2 diabetes mellitus, without relapses after the start of mepolizumab, at 4 weeks eosinophil reduction. Systemic corticosteroid was withdrawn.