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Pneumonia, eosinophilic

Eosinophilic pneumonia (EP) is a disease in which a certain type of white blood cell called an eosinophil accumulates in the lung. These cells cause disruption of the normal air spaces (alveoli) where oxygen is extracted from the atmosphere. Several different kinds of eosinophilic pneumonia exist and can occur in any age group. The most common symptoms include cough, fever, difficulty breathing, and sweating at night. EP is diagnosed by a combination of characteristic symptoms, findings on a physical examination by a health provider, and the results of blood tests and x-rays. Prognosis is excellent once most EP is recognized and treatment with corticosteroids is begun. more...

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Types of eosinophilic pneumonia

Eosinophilic pneumonia is divided into different categories depending upon whether a cause can be determined or not. Known causes include certain medications or environmental triggers, parasitic infections, and cancer. EP can also occur when the immune system attacks the lungs, a disease called Churg-Strauss syndrome. When a cause can not be found, the EP is labeled "idiopathic." Idiopathic EP can be divided into "acute eosinophilic pneumonia" (AEP) and "chronic eosinophilic pneumonia" (CEP) depending on the symptoms a person is experiencing.

Symptoms

Most causes of eosinophilic pneumonia have similar symptoms. Cough, fever, increasing breathlessness, and night sweats are prominent and almost universal. Acute eosinophilic pneumonia typically follows a rapid course. Fever and cough may develop only one or two weeks before difficulties breathing progress to the point of respiratory failure requiring mechanical ventilation. Chronic eosinophilic pneumonia usually follows a slower course. Symptoms accumulate over several months and include fevers, cough, breathlessness, wheezing, and weight loss. Individuals with CEP are often diagnosed with asthma before CEP is finally recognized.

EP due to medications or environmental exposures is similar and occurs after an exposure to a known offending agent. EP due to parasitic infections has a similar prodrome in addition to a host of different symptoms related to the variety of underlying parasites. EP in the setting of cancer often develops in the context of a known diagnosis of lung cancer, cervical cancer, etc.

Pathophysiology

Eosinophilic pneumonia can develop in several different ways depending on the underlying cause of the disease. Eosinophils are thought to play a central role in defending the body against infection by parasites. Many diseases, such as asthma and eczema, are caused when eosinophils overreact to environmental triggers and release an excess of chemicals (cytokines) such as histamine. The common characteristic among different causes of EP is eosinophil overreaction or dysfunction in the lung.

Medications and environmental exposures

Medications, drugs of abuse, and environmental exposures may all trigger eosinophil dysfunction. Medications such NSAIDs (ie ibuprofen), nitrofurantoin, phenytoin, L-tryptophan, and ampicillin and drugs of abuse such as inhaled heroin and cocaine may trigger an allergic response which results in EP. Chemicals such as sulfites, aluminum silicate, and cigarette smoke can cause EP when inhaled. A New York City firefighter developed EP after inhalation of dust from the World Trade Center on September 11, 2001.

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Eosinophilic pneumonia as an initial manifestation of rheumatoid arthritis
From CHEST, 9/1/04 by Daniel Norman

Rheumatoid arthritis is a systemic disease that may have pulmonary manifestations. We describe a ease of eosinophilic pneumonia as the primary presentation of rheumatoid arthritis. While several cases of acute and chronic eosinophilic pneumonia have been reported in patients with preexisting rheumatoid arthritis, this is the first ease reported in which the eosinophilic lung disease was the initial manifestation of systemic rheumatoid arthritis.

Key words: eosinophilic pneumonia; pulmonary; rheumatoid arthritis

**********

Eosinophilic pneumonias are characterized by the presence of cough, lung infiltrates, and pulmonary eosinophilia. There are two forms of eosinophilic pneumonia, acute and chronic. The latter has a slower onset, milder fevers, and less hypoxia than the acute form) Both tend to respond promptly to therapy with corticosteroids, although the chronic form often recurs once treatment is discontinued. While a majority of patients who develop these pneumonias have a history of atopy, the etiologies of these disorders are unknown. These pneumonias are typically diagnosed by clinical features, by the exclusion of underlying infection or drag reaction, and by finding parenchymal infiltration with eosinophils by transbronchial biopsy or wedge resection. We describe here a case of eosinophilic pneumonia as the initial presentation of a patient with rheumatoid arthritis.

CASE REPORT

A 39-year-old woman presented to her internist with a 5 day history of a hacking, dry cough. Despite initial treatment with albuterol and guaifenesin, followed by a 6 day course of erythromycin, her symptoms progressed to include night sweats, fatigue, pleuritic chest pain, and severe arthralgias and myalgias. A chest radiograph revealed a right upper lobe infiltrate that was suggestive of pneumonia, and after a 12-day course of treatment with levofloxacin failed to improve the symptoms, the patient was referred to a pulmonologist for further evaluation. Her medical history was notable for a positive purified protein derivative test result and 12 months of treatment with isoniazid in childhood. The medications used were albuterol, guaifenesin, levofloxacin, and an oral contraceptive.

On presentation, the patient was ill-appearing, with a frequent. dry cough. She had a respiratory rate of 16 breaths/min, a temperature of 98.3[degrees]F, and bronchial breath sounds with egophony in the right upper and middle lung zones. There was no rash or palpable lymphadenopathy. The peripheral WBC count was 18,700 cells/[micro]L (neutrophils, 88%; lymphocytes, 8%; monocytes, 3%; eosinophils, 1%). As polyarthralgias progressed to polyarthritis, a rheumatologic workup revealed a sedimentation rate if 116, negative Lyme serology, positive antinuclear antibody (ANA) test result (titer, 1:40 with a diffuse pattern), negative anti-double strand DNA test result, and a markedly positive serum rheumatoid factor test result (titer, > 1:1,280). A chest CT scan (Fig 1) demonstrated dense consolidations in the posterior segment of the right upper lobe and superior segment of the right lower lobe, with air bronchograms and evidence of old granulomatous disease. Bronchoscopy revealed no endobronchial lesions or significant secretions, and the findings of BAL fluid cytology and cultures (including those for acid fast bacilli) were negative. Serology findings were negative fur coccidiomycosis, histoplasmosis, blastomycosis, parvovirus, pertussis, and mycoplasma pneumoniae, as was that for the anti neutrophil cytoplasmic antibody test. The patient was referred for video-assisted thoracoscopy for the tissue diagnosis of refractory pneumonia.

[FIGURE 1 OMITTED]

Wedge resection from the patient's right upper and lower lobes (Fig 2) revealed organizing pneumonitis with a prominent component of fibrinous exudates and numerous eosinophils, suggesting the presence of eosinuphilic pneumonia. The findings of tissue cultures, including acid-fast bacilli, were negative. The patient was started on a course of treatment with high-dose IV corticosteroids (initially 80 mg methylprednisolone daily), and her fever and pulmonary symptoms resolved within 4 days.

In the year since her presentation, the patient has not had a recurrence of her pulmonary: symptoms or infiltrates, but has had persistent polyarthritis despite several months of therapy with oral corticosteroids, rofecoxib, and hydroxychloroquine. Her course of treatment with corticosteroids was tapered off over a 6-month period, and she has been transitioned to therapy with methotrexate and etanercept to control her rheumatoid arthritis, with close follow-up given to her history of a positive purified protein derivative test result.

DISCUSSION

Rheumatoid arthritis is a systemic autoimmune disease associated with a number of pulmonary manifestations, including pleural disease, rheumatoid nodules, interstitial lung disease, pulmonary vascular disease, and airway involvement. (2) There have been a handful of ease reports in the English literature of acute eosinophilic pneumonia (one case) (3) and chronic eosinophilic pneumonia (five cases) (4-7) associated with rheumatoid arthritis (with varying degrees of peripheral eosinophilia and rheumatoid factor positivity). Many of these reports concerned patients with long-standing rheumatoid arthritis, in whom the medications used in the treatment of the arthritis may have contributed to the development of lung disease. In the most recent report, a 55-year old woman developed rheumatoid arthritis with peripheral eosinophilia coincident with the onset of eosinophilic pneumonia. (7) We describe the first report of a patient with rheumatoid arthritis, without peripheral eosinophilia, whose initial presentation was eosinophilic pneumonia. Although difficult to characterize as either acute or chronic eosinophilie pneumonia, this case is most consistent with the chronic variety,, given the subacute presentation, mild hypoxemia, and dense consolidations seen on chest imaging. However, in the year since her initial presentation our patient has not had any relapses of eosinophilic pneumonia, which is a common feature of the chronic subtype. This may be due to the uninterrupted use of immunosuppressive medications for the treatment of her persistent arthritic symptoms.

CONCLUSION

Several cases of eosinophilic pneumonia associated with rheumatoid arthritis have been described. We now include the ease of this patient wit h eosinophilic pneumonia as the initial presentation of the disease. Eosinophilic pneumonia should be considered as a diagnosis in a patient who presents with pulmonary infiltrates, fever, and joint symptoms consistent with rheumatoid arthritis.

REFERENCES

(1) Hayakawa H, Sate A, Toyoshima M, et al. A clinical study of idiopathic eosinophilic pneumonia. Chest 1994; 105:1462-1466

(2) Tanoue LT. Pulmonary manifestations of rheumatoid arthritis. Clin Chest Med 1998; 19:667-685

(3) Mehandru S, Smith RL, Sidhu GS, et al. Migratory pulmonary infiltrates in a patient with rheumatoid arthritis. Thorax 2002; 57:465-467

(4) Cooney TP. Interrelationship of chronic eosinophilic pneumonia, bronchiolitis obliterans, and rheumatoid disease: a hypothesis. J Clin Pathol 1981; 34:129-137

(5) Papiris SA, Maniati MA, Kalousis JV, et al. Chronic eosinophilic pneumonia in rheumatoid arthritis. Monaldi Arch Chest Dis 1995; 50:360-362

(6) Payne CR, Connelhm SJ. Chronic cosinophilie pneumonia complicating long-standing rheumatoid arthritis. Postgrad Med J 1980; 56:519-520

(7) Kwak JJ, Chang JE, Lee J, et al. Chronic eosinophilic pneumonia associated with an initiation of rheumatoid arthritis. Clin Rheumatol 2003; 22:240-243

* From the Divisions of Pulmonary, Critical Care, and Sleep Medicine (Drs. Norman and Roberts), and Rheumatology (Dr. Piecyk), Beth Israel Deaconess Medical Center, Boston, MA. Manuscript received December 2, 2003; revision accepted May 26, 2004.

Reproduction of dais article is prohibited without written permission from the American College of Chest Physicians (e-mail: permissions@ehestnet.org).

Correspondence to: David Roberts, MD, FCCP, Division of Pulmonary, Critical Care, and Sleep Medicine (1), Beth Israel Deaconess Medical Center, E/KS-B23, 330 Brookline Ave, Boston, MA 02215; e-mail: dhrohert@bidmc.harvard.edu

COPYRIGHT 2004 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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