Chest
Volume 103, Issue 1, January 1993, Pages 162-165
Journal home page for Chest

Clinical Investigations
Chronic Eosinophilic Pneumonia: A Long-term Follow-up of 12 Patients

https://doi.org/10.1378/chest.103.1.162Get rights and content

Chronic eosinophilic pneumonia (CEP) is a rare disorder of unknown etiology characterized by striking systemic and pulmonary manifestations such as fever, weight loss, blood eosinophilia, characteristic fluffy peripheral opacities on chest radiograph, and a prompt response to corticosteroid therapy. While the initial phase has been well documented, there is very limited information concerning the long-term natural history and treated course of this condition. We report the clinical and laboratory findings together with the long-term follow-up data on 12 patients with classic CEP who were followed up for a mean of 10.2 years (range, 4 to 13 years). The most striking feature of the long-term follow-up was the occurrence of relapses of CEP (often on multiple occasions) when corticosteroid therapy was discontinued or the dose was tapered. In those nine patients in whom steroid withdrawal was commenced, there was a clinical, hematologic, and radiologic relapse in seven (58 percent). However, prompt reinstitution of therapy led to a rapid resolution of symptoms. By contrast, two patients (17 percent) showed no evidence of relapse when steroid therapy was discontinued. A further three patients (25 percent) are maintained on a regimen of low-dose steroid therapy with no episodes of relapse. Reassuringly, all 12 patients are well at the end of a long period of follow-up. These data suggest that the long-term prognosis for patients with CEP is excellent but the majority will require long-term low-dose oral corticosteroid therapy in order to prevent relapse.

Section snippets

METHODS

Twelve patients diagnosed as having CEP and currently attending the respiratory clinic at St. Vincent's Hospital, Dublin, Ireland, were reviewed. The diagnosis was based on classic symptoms, blood eosinophilia, characteristic radiographic findings, and a rapid response to corticosteroid therapy. Parasitic infestations were excluded in all cases and no patient had ingested any drug known to produce a blood eosinophilia. No patient had vasculitis or a malignant neoplasm at diagnosis nor did any

Clinical

These data are summarized in Table 1. All patients were female. All 12 had been well before presentation apart from four who had mild asthma for 1, 5, 8, and 23 years, respectively, before the onset of the present illness. One patient had nasal polyps removed in the past and all but one (case 6) were nonsmokers. The presenting complaints were as follows: cough/sputum (ten patients), weight loss (eight patients), wheeze (six patients), dyspnea (six patients), night sweats (six patients), and

DISCUSSION

Chronic eosinophilic pneumonia is a distinct clinical entity, the classic presenting features having been first described by Carrington et al1 in 1969 when they reported on nine patients with this rare disorder. The early course of this illness has now been well documented,1, 2, 3, 4, 5, 6 but the long-term outcome has not been defined. There have been only two intermediate follow-up studies of this disorder.2, 3 Pearson and Rosenow2 followed up eight patients for an average of 6 years while

REFERENCES (6)

  • CB Carrington et al.

    Chronic eosinophilic pneumonia

    N Engl J Med

    (1960)
  • DJ Pearson et al.

    Chronic eosinophilic pneumonia (Carrington's): a follow-up study

    Mayo Clin Proc

    (1978)
  • PJ Jederlinic et al.

    Chronic eosinophilic pneumonia—a report of 19 cases and a review of the literature

    Medicine

    (1988)
There are more references available in the full text version of this article.

Cited by (0)

View full text