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Idiopathic chronic eosinophilic pneumonia (CEP) is a rare disease of unknown cause characterized by eosinophilic alveolar and interstitial infiltration. The authors describe the case of a 46-year-old black man, presenting with insidious onset and progressive course of dyspnea on minimum exertion, cough, fever, night sweats, and weight loss for one year and worsening in the last three months. The main findings were serum eosinophilia. Chest radiographs showed multifocal infiltrations of irregular distribution in both lungs and a restrictive functional impairment. The patient underwent open lung biopsy, and the anatomopathological examination revealed consolidation by exudate constituted predominantly by macrophages (25%) and eosinophils (51%), which filled small air spaces, including respiratory and membranous bronchioles. The anatomopathological diagnosis was eosinophilic pneumonia (eosinophils > 25% is widely accepted for diagnosing eosinophilic pneumonia). The patient had a good clinical response after starting corticosteroid therapy.

Eine 43-jahrige Patientin mit den klinischen Zeichen eines fieberhaften respiratorischen Infekts wurde trotz mehrfacher Antibiotikatherapie mit persistierenden beidseitigen pulmonalen Infiltraten stationar eingewiesen. In der weiteren Diagnostik fiel im Diffentialblutbild eine Eosinophilie von 31% auf. In der Bronchoskopie mit bronchoalveolarer Lavage wurde ebenfalls eine Eosinophilie gesehen. Nach Ausschluss bekannter Ursachen einer pulmonalen Eosinophilie wurde die Diagnose einer chronischen idiopathischen eosinophilen Pneumonie gestellt. Unter alleiniger Therapie mit hochdosierten Kortikosteroiden normalisierten sich die pathologischen Laborwerte, die klinischen und die radiologischen Befunde innerhalb 1 Woche annahernd vollstandig. Bei therapierefraktaren pulmonalen Infiltraten sollten die Diagnose einer akuten oder chronischen eosinophilen Pneumonie in Betracht gezogen und eine Bronchoskopie mit bronchoalveolarer Lavage veranlasst werden. Differentialdiagnostisch muss aus prognostischen und therapeutischen Grunden zwischen einer akuten und chronischen idiopathischen eosinophilen Pneumonie unterschieden werden. Ein rasches Ansprechen auf Kortikosteroide unterstutzt die Diagnose. Bei einer chronischen idiopathischen eosinophilen Pneumonie ist auf eine Therapiedauer von etwa 6 Monaten zur Vermeidung von Rezidiven zu achten.

la pneumonie chronique idiopathique a eosinophiles ou maladie de Carrington est une maladie rare de cause inconnue, caracterisee par l'association de symptomes respiratoires et generaux subaigus ou chroniques, une eosinophilie alveolaire et/ou sanguine et des infiltrats pulmonaires de topographie peripherique en imagerie. Il s'agit d'une pathologie benigne d’evolution favorable. Nous rapportons un cas de cette pathologie rare dans sa forme pseudotumorale de diagnostic difficile caracterise par l'absence d’eosinophilie sanguine et alveolaire et d’evolution fatale. Nous rappellerons aussi les caracteres cliniques, radiologiques, therapeutiques et evolutifs de la maladie de Carrington a travers une revue de litterature.

Idiopathic chronic eosinophilic pneumonia (ICEP) is characterized by subacute or chronic respiratory and general symptoms, alveolar and/or blood eosinophilia, and peripheral pulmonary infiltrates on chest imaging. Eosinophilia is present in most cases, usually in excess of 1000/mm3. In absence of significant blood eosinophilia, a diagnosis of ICEP is supported by the demonstration of bronchoalveolar lavage eosinophilia. ICEP is typically associated with eosinophil counts higher than lymphocyte counts in the bronchoalveolar lavage. ICEP is a rare disorder of unknown cause. Its exact prevalence remains unknown. ICEP may affect every age group but is rare in childhood. It is twice as frequent in women as in men. One third to one half of the ICEP patients have a history of asthma. The mainstay of treatment of ICEP is systemic corticosteroids. Response to oral corticosteroid therapy is dramatic and has led to the consideration of corticosteroid challenge as a diagnostic test for ICEP. Nevertheless, relapses or development of severe asthma are frequent when tapering or withdrawing treatment. Long-term oral corticosteroid therapy is necessary in up to half of the patients.

Hypereosinophilic asthma (defined by blood eosinophils >1 G/l, especially >1.5 G/l) may occur as a primary pathology (and often difficult to treat) or as the manifestation of a hypereosinophilic disorder of determined cause (iatrogenic, parasitic, allergic bronchopulmonary aspergillosis) or idiopathic (idiopathic chronic eosinophilic pneumonia, Churg–Strauss syndrome, idiopathic hypereosinophilic syndrome).

A 34-year-old man with bronchial asthma was introduced to our clinic because his blood tests showed a white blood cell count of 25,200/μL with a differential of 70% eosinophils, and chest computed tomography revealed non-segmental multi-lobar consolidation. Bronchoscopy was performed and yellow broncho-alveolar lavage fluid (BALF) was collected (Picture 1). The supernatant of BALF was still yellow (Picture 2). The total cell count (TCC) was 110×105/mL and the cell differentiation of BALF showed a significant elevation of eosinophils (98%). As a result, a diagnosis of idiopathic chronic eosinophilic pneumonia (EP) was made. Eosinophilic cationic protein (ECP), a representative eosinophil-derived protein, in BALF was also significantly elevated (105 μg/L).

International audience; Since idiopathic chronic eosinophilic pneumonia (ICEP) and asthma are frequently associated, their possible reciprocal influence on clinical presentation and evolution were investigated. The clinical and follow-up features of 53 cases of ICEP, of which 41 (77%) had asthma, were reviewed retrospectively. Asthma preceded the diagnosis of ICEP in 26 patients, was contemporaneous in eight patients, and developed 17+/-12 months after ICEP in seven patients. Presentation of ICEP was similar in asthmatics and nonasthmatics with the exception of a higher level of total immunoglobulin E in the former group. Patients with asthma at the time of diagnosis of ICEP were more likely to remain free of relapse of ICEP (56 versus 23%) and had a lower number of relapses per year of follow-up (median 0 versus 0.24). Moreover, they were treated more frequently with long-term inhaled corticosteroids (88 versus 31%) at last follow-up. Asthma got worse after the diagnosis of ICEP and frequently required long-term oral corticosteroids. To conclude, among patients with idiopathic chronic eosinophilic pneumonia asthmatics have a lower frequency of relapse than nonasthmatics, possibly because of a higher use of inhaled corticosteroids. The occurrence of idiopathic chronic eosinophilic pneumonia in asthmatics is often associated with the development of severe asthma.

Two cases of pulmonary eosinophilia associated with coccidioidal infections are reported. Pulmonary eosinophilia in these cases represents a hypersensitivity reaction to the fungus. Histologically, the pulmonary eosinophilia in these cases closely mimicked or appeared identical to idiopathic chronic eosinophilic pneumonia. Coccidioides immitis organisms were rare or absent in the areas of pulmonary eosinophilia. Recognition of this phenomenon is important for proper care of the patient.