(Chest. 2001;119:968-970.)
© 2001
American College of Chest Physicians
Chronic Eosinophilic Pneumonia Presenting With Recurrent Massive Bilateral Pleural Effusion*
Case Report
Yaseen S. Samman, MD, FCCP;
Siraj O. Wali, MD, FCCP;
Mohammed A. Abdelaal, MD;
Mohsin T. Gangi, MD and
Ayman B. Krayem, MD
*
From the Department of Medicine, King Khalid National Guard Hospital, Jeddah, Saudi Arabia.
Correspondence to: Yaseen S. Samman, MD, FCCP, Department of Medicine, King Khalid National Guard Hospital, PO Box 9515, Jeddah 21423, Saudi Arabia; e-mail: Sammanys{at}ngha.med.sa
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Abstract
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We describe a rare case of a 29-year-old woman with chronic
eosinophilic pneumonia (CEP) presenting with massive bilateral pleural
effusion leading to respiratory failure, a complication that was not
reported before with CEP. The patient was successfully managed with
ventilatory support and steroid therapy. On long-term follow-up, she
remained well, receiving a low maintenance dose of prednisone without
evidence of relapse of the disease.
Key Words: chronic eosinophilic pneumonia • massive pleural effusion • respiratory failure.
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Introduction
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Chronic
eosinophilic pneumonia (CEP), a rare eosinophilic lung disease of
unknown etiology, is characterized by peripheral blood eosinophilia,
chest radiograph infiltrates, and prompt response to corticosteroid
therapy.1
The first detailed description of CEP was
by Carrington et al in 1969.2
CEP most commonly affects women of middle age. The usual symptoms
are cough, dyspnea, fever, and weight loss. We report a case of CEP
presenting with massive, rapidly accumulating pleural effusion
progressing to respiratory failure that, to our knowledge, has not been
reported previously.
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Case Report
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A 29-year-old nonsmoking woman presented with a 5-month history
of shortness of breath and dry cough. She denied any history of fever,
night sweats, or weight loss. She had no history of chest pain,
palpitation, arthralgia, arthritis, or skin rash. There was no
neurologic or GI symptoms and no travel history.
The patient was initially admitted to another facility with the
same symptoms and received a diagnosis of bronchial asthma and
bilateral exudative pleural effusions requiring frequent uncomplicated
therapeutic drainage. The original reported WBC was 10,000 cells/µL
with 70% eosinophils; no pulmonary function tests were performed.
However, pleural analysis was reported as clear and nonhemorrhagic,
with 70% lymphocytes, 1% neutrophils, and 2% eosinophils. Pleural
biopsy was reported to show nonspecific inflammatory reaction
consisting mainly of eosinophils.
On admission at our hospital, she was afebrile, with a
respiratory rate of 25 breaths/min and decreased chest expansion.
Dullness to percussion was noted with decreased air entry at the bases.
The rest of the physical examination was unremarkable. The patient was
receiving salbutamol and beclomethasone inhalers from the other
facility.
Results of laboratory investigations are as follows: hemoglobin, 13.9
g/dL; WBC count, 9,300 cells/µL; platelets, 360,000 cells/µL; and
persistent eosinophilia of 1,800 to 3,200 cells/µL. Basic biochemical
profile and serum immunoglobulins, including IgE were normal. Serology
for locally prevalent parasites (amebiasis, echinococcosis,
leishmaniasis, and schistosomiasis) by enzyme-linked immunosorbent
assay method was negative. Antinuclear antibody, antimitochondrial
antibody, anti-smooth muscle antibody, and anti-double–strand
DNA were negative. Rheumatoid factor was weakly positive. Repeated
sputum microscopy and culture and polymerase chain reaction for
Mycobacterium tuberculosis results were negative. Mantoux
test was negative. Stool examinations for ova and parasites (namely
trichinosis, echinococcus, and schistosomiasis) were negative.
Pulmonary function tests showed a severe restrictive pattern (Table 1
).
Chest radiography revealed bilateral pleural effusions with
interstitial and airspace opacities in the lower third of the lungs
(Fig 1 ). A CT scan of the chest demonstrated bilateral loculated pleural
effusions; collapse and consolidation of the right lower, middle, and
lingula lobes; and ground-glass opacities in the right upper lobe (Fig 2
). The pleural fluid protein was 4.3 g/dL, lactate dehydrogenase was
1,554 IU/L, and total WBC count was 1,580 cells/µL with 21%
eosinophils and no abnormal cells. Pleural biopsy showed mixed
inflammatory cell infiltrate with predominance of eosinophils.
Echocardiographic findings were normal. Bone marrow examination showed
40% infiltration with eosinophils and a normal karyotype.
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Figure 2.. CT scan of the chest at the level of the carina
showing bilateral pleural effusion with collapse and consolidation of
the right lower lobe and middle lobe and ground-glass opacity in the
right upper lobe.
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The patient required repeated therapeutic pleural fluid drainage
and insertion of a chest tube without clinical improvement because of
rapid reaccumulation and loculation of the fluid. Respiratory failure
occurred on day 3, and she required intubation and mechanical
ventilation. Bronchoscopy demonstrated normal airways, and BAL revealed
a WBC count of 5,100 cells/µL with 80% eosinophils and a negative
culture finding.
On the basis of the persistent peripheral eosinophilia,
infiltrates on chest radiography, eosinophilic pleural effusion, high
percentage of eosinophils in the BAL, and negative investigations for
other causes of eosinophilia, the diagnosis of CEP was made.
The patient responded rapidly to treatment with IV methylprednisolone,
1 g/d; 3 days later, she was successfully extubated. Her treatment was
changed to oral prednisolone, 40 mg/d, and she was discharged on the
eighth hospital day.
On follow-up visits, the patient remained asymptomatic, and the
prednisolone was tapered to 15 mg/d by 6 months and was stopped by 9
months, with chest radiography showing residual bilateral pleural
thickening, and significant improvement on pulmonary function testing
(Table 1)
. On follow-up, there was no relapse 4 months after
discontinuation of steroid therapy.
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Discussion
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Our patient satisfied the criteria for the diagnosis of
CEP.1
Pulmonary involvement may occur in 40% of patients
with idiopathic hypereosinophilic syndrome (HES) and may therefore
closely resemble CEP.1
3
However, because of the absence
of multiorgan involvement with signs of end-organ damage, one of the
triad of HES,3
and the characteristic radiologic findings
of CEP in our patient, the diagnosis of HES was excluded. Bronchiolitis
obliterans with organizing pneumonia may also mimic CEP, but the
radiologic and BAL findings were incompatible with the diagnosis of
bronchiolitis obliterans with organizing pneumonia.1
The onset of CEP is usually insidious, and symptoms are
present for at least a few months before diagnosis, as in our patient.
If the diagnosis is delayed, progression to respiratory failure may
occur.1
Bronchial asthma is present in about half of the
cases.4
Peripheral eosinophilia occurs in most patients
with CEP and may be associated with elevated IgE levels. The latter may
parallel the disease activity.1
In 25% of patients with
CEP, chest radiography shows the characteristic, extensive bilateral
peripheral infiltrates, described as a photographic negative image of
pulmonary edema.5
This was not present in our
patient.
Pleural effusion is uncommon in CEP. In a review of 19 cases of
CEP, only 2 patients had pleural effusion, and in a multicenter study,
2 of 62 patients had bilateral small-size pleural
effusions.4
6
The mechanism of pleural effusion in CEP is
unknown. However, it might be because of tissue damage induced by
infiltration with eosinophils, antibody-mediated cellular toxicity, or
the release of intracytoplasmic leukotriene that increases
microvascular permeability.1
Our patient had severe restrictive lung disease, as reported in
the majority of patients with CEP.1
Durieu et
al7
found that 9 of 19 patients with CEP had restrictive
abnormalities and 4 patients had obstructive abnormalities, and on
long-term follow-up, 8 of the 19 patients showed complete recovery. The
high diffusion capacity of the lung for carbon monoxide/alveolar
volume ratio in our patient could be explained, initially, by the large
pleural effusion, collapse and entrapment of the lungs, and, lately, by
pleural thickening.7
8
9
Open lung biopsy is performed when the diagnosis is in
doubt,1
and this usually shows interstitial and alveolar
infiltration with eosinophils, microabscesses, Charcot-Leyden crystals,
and bronchiolitis obliterans. The prompt response to corticosteroids,
as seen in our patient, is characteristic in CEP.1
The
prognosis of CEP is excellent, but there may be relapse of disease.
Therefore, decreasing doses of corticosteroids are recommended for at
least 6 to 12 months.1
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Conclusion
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Although not reported before, rapidly accumulating pleural
effusions could be a presenting feature of CEP. Early recognition of
this is important in view of the good response to treatment with
corticosteroids.
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Footnotes
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Abbreviations: CEP = chronic eosinophilic
pneumonia; HES = hypereosinophilic syndrome
Received for publication November 23, 1999.
Accepted for publication August 15, 2000.
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References
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Allen, JN, Davis, WB (1994) Eosinophilic lung diseases. Am J Respir Crit Care Med 150,1423-1438[ISI][Medline]
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Carrington, CB, Addington, WW, Goff, AM, et al (1969) Chronic eosinophilic pneumonia. N Engl J Med 280,787-798
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Fauci, AS, Harley, JB, Roberts, WC, et al (1982) The idiopathic hyperoesinophilic syndrome: clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med 97,78-92
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Marchand, E, Reynaud-Gaubert, M, Lauque, D, et al (1998) Idiopathic chronic eosinophilia pneumonia: a clinical and follow-up study of 62 cases. Medicine 77,299-312[CrossRef][Medline]
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Gaensler, EA, Carrington, CB (1977) Peripheral opacities in chronic eosinophilic pneumonia: the photographic negative of pulmonary edema. AJR Am J Roentgenol 128,1-13[Abstract]
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Jederlinic, PJ, Sicilian, L, Gaensler, EA (1988) Chronic eosinophilic pneumonia: a report of 19 cases and a review of the literature. Medicine 67,154-162[Medline]
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Durieu, J, Wallaert, B, Tonnel, AB (1997) Long term follow-up of pulmonary function in chronic eosinophilic pneumonia. Eur Respir J 10,286-291[Abstract]
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Seigler, D, Zorab, PA (1982) The influence of lung volume on gas transfer in scoliosis. Br J Dis Chest 76,44-50[ISI][Medline]
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Miller, A (1986) Pulmonary function tests in clinical and occupational lung disease. ,147-148 Grune and Stratton Orlando, FL.
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Abstract
Introduction
