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Pneumopatia eosinofílica crônica idiopática
ORPHA:2902CID-10 · J82CID-11 · CB02.11DOENÇA RARA

Doença pulmonar intersticial muito rara, grave, de início insidioso, com manifestações respiratórias inespecíficas subagudas ou crónicas (dispneia, tosse, sibilos) frequentemente associadas a manifestações sistémicas (fadiga, mal-estar, perda de peso).

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Introdução

O que você precisa saber de cara

📋

Doença pulmonar intersticial muito rara, grave, de início insidioso, com manifestações respiratórias inespecíficas subagudas ou crónicas (dispneia, tosse, sibilos) frequentemente associadas a manifestações sistémicas (fadiga, mal-estar, perda de peso).

Publicações científicas
59 artigos
Último publicado: 2026 Feb

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: J82
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫁
Pulmão
5 sintomas
🩸
Sangue
3 sintomas
🧬
Pele e cabelo
2 sintomas
🫃
Digestivo
1 sintomas
🛡️
Imunológico
1 sintomas
📏
Crescimento
1 sintomas

+ 14 sintomas em outras categorias

Características mais comuns

90%prev.
Achado anormal em imagem torácica pulmonar
Muito frequente (99-80%)
90%prev.
Consolidação parenquimatosa
Muito frequente (99-80%)
55%prev.
Concentração elevada de proteína C-reativa circulante
Frequente (79-30%)
55%prev.
Pneumonite por hipersensibilidade
Frequente (79-30%)
55%prev.
Hipoxemia
Frequente (79-30%)
55%prev.
Dermatite atópica
Frequente (79-30%)
27sintomas
Muito frequente (2)
Frequente (16)
Ocasional (5)
Muito raro (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.

Achado anormal em imagem torácica pulmonarAbnormal pulmonary thoracic imaging finding
Muito frequente (99-80%)90%
Consolidação parenquimatosaParenchymal consolidation
Muito frequente (99-80%)90%
Concentração elevada de proteína C-reativa circulanteElevated circulating C-reactive protein concentration
Frequente (79-30%)55%
Pneumonite por hipersensibilidadeHypersensitivity pneumonitis
Frequente (79-30%)55%
HipoxemiaHypoxemia
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico59PubMed
Últimos 10 anos29publicações
Pico20236 papers
Linha do tempo
2026Hoje · 2026🧪 2008Primeiro ensaio clínico📈 2023Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Pneumopatia eosinofílica crônica idiopática

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

1 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
29 papers (10 anos)
#1

Carrington's Disease: A Case Report.

Cureus2026 Feb

Idiopathic chronic eosinophilic pneumonia (ICEP), also known as Carrington's disease, is a rare eosinophilic lung disorder characterized by pulmonary infiltrates with no identifiable cause. We report the case of a 59-year-old man with no significant medical history who presented with an eight-month history of progressive exertional dyspnea and a non-productive cough. Chest imaging showed bilateral peripheral ground-glass opacities. Laboratory evaluation revealed peripheral blood eosinophilia (2,000/mm³), and bronchoalveolar lavage demonstrated eosinophilic alveolitis (30%). The patient was treated with oral corticosteroids, resulting in complete symptom resolution and marked radiological improvement.

#2

Six-Month Benralizumab Maintenance for Relapsing Chronic Eosinophilic Pneumonia Guided by Eosinophil Kinetics.

Respirology case reports2026 Feb

Idiopathic chronic eosinophilic pneumonia (ICEP) often relapses upon corticosteroid tapering. Biologics targeting interleukin-5 (IL-5) are effective, but optimal dosing intervals remain unclear. We report a case of relapsing ICEP in a patient in her 50s. Mepolizumab, an IL-5 ligand blocker, failed to maintain remission, with clinical relapse occurring 4 months after initiation. Switching to benralizumab, an interleukin-5 receptor blocker, induced rapid and deep eosinophil depletion. For optimising dosing, treatment was temporarily withheld, revealing a prolonged remission duration of 8 months before eosinophil recovery and clinical relapse. Based on these kinetics, a 6-monthly benralizumab maintenance strategy was established. The patient has remained relapse-free with zero eosinophils for over 2 years under this biannual regimen. This case suggests that benralizumab offers superior durability compared to mepolizumab in CEP due to deep depletion, enabling an extended, cost-effective dosing interval guided by individual eosinophil recovery kinetics.

#3

Idiopathic chronic eosinophilic pneumonia presenting as diffuse alveolar haemorrhage.

The National medical journal of India2025

Eosinophilic pneumonia is rare and characterized by excessive accumulation of eosinophils in the alveolar macrophages and interstitium. The presentation can be acute or chronic. The patient can also present with respiratory failure requiring intensive support. These patients may have bronchial asthma at presentation or may be diagnosed with the same later. Diffuse alveolar haemorrhage, a rare complication of idiopathic chronic eosinophilic pneumonia, is life-threatening requiring urgent and aggressive investigation and management. We report a young male who had pneumonia and haemoptysis and was diagnosed to have idiopathic chronic eosinophilic pneumonia and diffuse alveolar haemorrhage.

#4

Rapid Desensitization to Reslizumab in an 80-Year-Old Woman With Chronic Eosinophilic Pneumonia, Severe Persistent Asthma, and Chronic Urticaria.

Cureus2025 Nov

Our patient is an 80-year-old woman with chronic eosinophilic pneumonia (CEP), chronic urticaria (CU), severe persistent asthma (SPA), long-term oral corticosteroid therapy, and a resected papillary thyroid carcinoma. The patient had normal, stable immunoglobulin E (IgE) levels across all treatments ranging from 16 to 37 IU/mL. In treating her CEP and severe asthma, she trialed monoclonal antibody therapy with mepolizumab, benralizumab, dupilumab, and omalizumab, which were each begun and then discontinued successively due to severe side effects or allergic reaction. However, reslizumab offered 10 months of notable relief from urticaria and dyspnea without oral corticosteroids but was discontinued after the patient developed infusion-associated urticaria. Given her relief with reslizumab, we planned to desensitize her to this medication via a stepwise, slowed infusion up to a target therapeutic dose. Complete vital signs and physical exam looking for symptoms of an allergic response, including angioedema, urticaria, dyspnea, and nausea, were performed every 15 minutes. Our patient was successfully desensitized to reslizumab at a target dose of 195 mg over three hours. Vitals were checked every 15 minutes, which were within normal limits throughout. Further, she was monitored for any symptoms of allergic reaction, including angioedema, nausea, urticaria, and dyspnea, and denied any complaints throughout the procedure. Our patient reported notable relief of dyspnea and fatigue at two weeks and four weeks post-procedure. As opposed to a decrease in dose or discontinuation of treatment, patients may undergo rapid desensitization through a stepwise, slowed infusion rate to better tolerate a previously allergenic medication.

#5

The clinical question of mepolizumab in the long-term treatment of idiopathic chronic eosinophilic pneumonia; how long should we use mepolizumab?

Respiratory medicine case reports2024

The efficacy of mepolizumab as an alternative to glucocorticoids for treating idiopathic chronic eosinophilic pneumonia (ICEP) has been reported. However, various questions remain unanswered, such as the most appropriate dose and dosage interval of mepolizumab for ICEP, how long efficacy is maintained, how long administration should be continued, and whether and when discontinuation can be considered. We present herein three cases of refractory ICEP treated with mepolizumab at a dose of 100 mg every 4 or 8 weeks. No recurrences were observed after 77 months of treatment in Case 1 and after 45 months in Case 2. Case 3 was treated with mepolizumab for 33 months, but ICEP relapsed 42 months after discontinuation of mepolizumab. In conclusion, mepolizumab for refractory ICEP should be continued for as long as possible, considering disease status, glucocorticoid-related adverse events, and the financial situation of the patient.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC31 artigos no totalmostrando 29

2026

Carrington's Disease: A Case Report.

Cureus
2026

Six-Month Benralizumab Maintenance for Relapsing Chronic Eosinophilic Pneumonia Guided by Eosinophil Kinetics.

Respirology case reports
2025

Idiopathic chronic eosinophilic pneumonia presenting as diffuse alveolar haemorrhage.

The National medical journal of India
2025

Rapid Desensitization to Reslizumab in an 80-Year-Old Woman With Chronic Eosinophilic Pneumonia, Severe Persistent Asthma, and Chronic Urticaria.

Cureus
2024

The clinical question of mepolizumab in the long-term treatment of idiopathic chronic eosinophilic pneumonia; how long should we use mepolizumab?

Respiratory medicine case reports
2024

Successful Treatment of Relapsing Chronic Eosinophilic Pneumonia With Mepolizumab: A Case Report.

Cureus
2024

The long-term outcomes of mepolizumab treatment at 100 mg dose on idiopathic chronic eosinophilic pneumonia: A real-life experience.

Allergy and asthma proceedings
2024

Monoclonal antibodies in idiopathic chronic eosinophilic pneumonia: a scoping review.

BMC pulmonary medicine
2023

Misdiagnosis asthma in adult, three rare causes.

La Tunisie medicale
2023

Eosinophilia with Lung Involvement in an Elderly Patient with a History of Chronic Obstructive Pulmonary Disease.

Journal of community hospital internal medicine perspectives
2023

Case Report: Mepolizumab in the treatment of idiopathic chronic eosinophilic pneumonia.

F1000Research
2023

Eosinophilic Lung Diseases.

Immunology and allergy clinics of North America
2023

Targeting IL-5/5R for the treatment of idiopathic chronic eosinophilic pneumonia.

The journal of allergy and clinical immunology. In practice
2023

The role of flexible bronchoscopy in the diagnostic pathway of children with unexplained peripheral eosinophilia.

Pediatrics international : official journal of the Japan Pediatric Society
2022

Idiopathic Chronic Eosinophilic Pneumonia Evolving to Pulmonary Fibrosis: A Retrospective Analysis.

Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
2022

Idiopathic chronic eosinophilic pneumonia: a differential diagnosis of lower respiratory tract infection.

BMJ case reports
2022

Anti-IL-5 Agents for the Treatment of Idiopathic Chronic Eosinophilic Pneumonia: A Case Series.

Journal of asthma and allergy
2021

Evaluating the relationship between high-resolution computed tomography findings and their extent in eosinophilic lung diseases with peripheral blood eosinophil level.

Polish journal of radiology
2021

Recurrent pneumonia with persistent peripheral eosinophilia: Idiopathic chronic eosinophilic pneumonia.

Clinical case reports
2021

Idiopathic Chronic Eosinophilic Pneumonia.

Cureus
2020

An unusual case of eosinophilic lung disease with multiple cyst formation.

Respiratory medicine case reports
2020

Mepolizumab: an alternative therapy for idiopathic chronic eosinophilic pneumonia with glucocorticoid intolerance.

Drugs in context
2020

Real-Life Study of Mepolizumab in Idiopathic Chronic Eosinophilic Pneumonia.

Lung
2018

Eosinophilic lung disease with plastic bronchitis and bronchiectasis in an adolescent male.

Clinical case reports
2018

Elevated Levels of Intelectin-1, a Pathogen-binding Lectin, in the BAL Fluid of Patients with Chronic Eosinophilic Pneumonia and Hypersensitivity Pneumonitis.

Internal medicine (Tokyo, Japan)
2017

Chronic eosinophilic pneumonia: Adjunctive therapy with inhaled steroids.

Respiratory medicine case reports
2016

Eosinophilic pneumonias in children: A review of the epidemiology, diagnosis, and treatment.

Pediatric pulmonology
2016

Platelet activation markers overexpressed specifically in patients with aspirin-exacerbated respiratory disease.

The Journal of allergy and clinical immunology
2015

[Pseudotumoral form of chronic eosinophilic pneumopathy of fatal outcome].

The Pan African medical journal
Ver todos os 31 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Carrington's Disease: A Case Report.
    Cureus· 2026· PMID 41841088mais citado
  2. Six-Month Benralizumab Maintenance for Relapsing Chronic Eosinophilic Pneumonia Guided by Eosinophil Kinetics.
    Respirology case reports· 2026· PMID 41710012mais citado
  3. Idiopathic chronic eosinophilic pneumonia presenting as diffuse alveolar haemorrhage.
    The National medical journal of India· 2025· PMID 41645984mais citado
  4. Rapid Desensitization to Reslizumab in an 80-Year-Old Woman With Chronic Eosinophilic Pneumonia, Severe Persistent Asthma, and Chronic Urticaria.
    Cureus· 2025· PMID 41393680mais citado
  5. The clinical question of mepolizumab in the long-term treatment of idiopathic chronic eosinophilic pneumonia; how long should we use mepolizumab?
    Respiratory medicine case reports· 2024· PMID 39635341mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:2902(Orphanet)
  2. MONDO:0017363(MONDO)
  3. GARD:1130(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q56013979(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Pneumopatia eosinofílica crônica idiopática
Compêndio · Raras BR

Pneumopatia eosinofílica crônica idiopática

ORPHA:2902 · MONDO:0017363
Prevalência
Unknown
Herança
Not applicable
CID-10
J82 · Eosinofilia pulmonar, não classificada em outra parte
CID-11
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0008680
Repurposing
47 candidatos
atovaquonemitochondrial electron transport inhibitor
azithromycinbacterial 50S ribosomal subunit inhibitor
benzylpenicillinpenicillin binding protein inhibitor
+17 outros
EuropePMC
Wikidata
Papers 10a
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