Chest
Volume 89, Issue 3, March 1986, Pages 435-439
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Review
Clinical Spectrum of Pulmonary Mucormycosis

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Pulmonary mucormycosis is an uncommon, but important, opportunistic fungal pneumonia which is often diagnosed post-mortem. This review emphasizes clinical and pathologic characteristics of pulmonary mucormycosis that differentiate this infection from other fungal pneumonias. The most common clinical presentation of pulmonary mucormycosis is a rapidly progressive pneumonia with diffuse infiltrates on chest radiographic examination of a patient with an underlying hematologic malignancy treated with immunosuppressive drugs. Other immunocompromised hosts at risk for pulmonary mucormycosis include patients with diabetes mellitus who may develop a distinctive endobronchial form of this disease. Early consideration of this diagnosis, along with aggressive diagnostic evaluation, are critical to effective therapy and patient survival. While treatment with amphotericin B is the mainstay of therapy for pulmonary mucormycosis, diabetics with endobronchial disease may benefit from early, aggressive surgical resection of the involved lung tissue.

Section snippets

Microbiology and Pathology

Mucorales are ubiquitous saphrophytic fungi found in soil or decaying organic matter. Three genera are described as human pathogens: Rhizopus, Absidia and Mucor. Production of spores, which become airborne, leads to the primary route of innoculation in man, the respiratory tract. The term Phycomycoses has been used in the past to describe infections caused by these organisms, but this term is no longer favored.6 Although the members of the family Mucorales belong to the class Phycomycetes,

Predisposing Factors

These saphrophytic fungi have rarely been reported to be pathogenic in normal people.10, 11, 12 However, pulmonary mucormycosis is clearly an infection that has increased with the development of modern medicine.2, 3, 4, 5 Numerous predisposing clinical factors have been described, including diabetes, diabetic ketoacidosis,5 corticosteroid therapy,4, 5, 6 leukemia, lymphoma,2 immunosuppressive therapy,2, 3, 4, 5 neutropenia,3 antibiotic therapy,4 salicylate-induced acidosis,13 elastoplast

Clinical Presentation

The most common presentation of invasive pulmonary mucormycosis occurs in patients with an underlying hematologic malignancy being treated with immunosuppresive or antimicrobial drugs.2, 3, 5, 6, 7, 8 These patients often present with an acute illness at their neutropenic nadir, consisting of cough, fever and variable sputum production. Pleuritic chest pain is common. Chest examination may demonstrate rales with evidence of pulmonary consolidation; pleural friction rubs are not uncommon.

Case Report

A 44-year-old man with a nine-month history of hairy cell leukemia was admitted to the hospital with fever, right-sided chest pain, and hemoptysis. He had been treated with chlorambucil 4 mg per day, but was not responding to therapy. On examination, he appeared acutely and chronically ill with respiratory distress. His temperature was 39°C; chest examination revealed findings of consolidation and a friction rub in the right lateral chest. Total white blood cell count was 300 cells/cu mm with

Discussion

Pulmonary mucormycosis may also develop in patients with poorly controlled diabetes mellitus and ketoacidosis with a clinical syndrome similar to patients with hematologic malignancies. Donohue20 has reported that there is an additional, distinct clinical syndrome of mucormycosis in diabetic patients. These patients have evidence of an endobronchial lesion with confinement of the lung pathology to a discrete area. Serious complications develop because the organism invades the airways and hilar

Case Report

A 59-year-old diabetic woman with a long history of asthma was hospitalized with ketoacidosis and an upper gastrointestinal hemorrhage. She had been treated with corticosteroid drugs intermittently in the past for control of her asthma; at the time of admission, she was on therapy with a tapering dose of prednisone. Physical examination revealed a woman who was afebrile with postural hypotension and guaiac positive material in a gastric aspirate. White blood cell count was 28,800/cu mm; serum

Comment

There are 11 reported cases in the literature of primary major airway involvement with invasive mucormycosis.20, 21, 22, 23, 24, 25, 26, 27 Nine of these patients were diagnosed antemortem, eight by bronchoscopic examination. Of the cases with specific pulmonary locations reported, seven of ten patients had lesions of the right lung and two had tracheal lesions. In nine patients diagnosed antemorten, five survived. Four of these five were managed by surgical resection. One case was treated

Radiographic Features

The radiographic presentation of pulmonary mucormycosis has been previously well reviewed.9, 28, 29 Reported chest radiographic abnormalities include nodular, lobar, or wedge-shaped infiltrates (Fig 2 and 3); mediastinal widening; bronchopneumonia; solitary nodule; miliary pattern; cavitation; fungus ball; and pleural effusions. The more typical findings on chest radiographic examination are those of vascular invasion by fungus. This may begin as a nodular or nonspecific acinar infiltrate which

Diagnosis

Antemortem diagnosis has been made infrequently in pulmonary mucormycosis because of the acute course of the illness, lack of consideration of the diagnosis, and the need for tissue to establish the diagnosis. Result of sputum culture is usually negative,5 but a positive culture for Mucorales from sputum is highly suggestive of invasive infection in the appropriate host.10 Definitive diagnosis, however, requires histologic demonstration of tissue invasion with characteristic broad, nonseptate

Differential Diagnosis

There are many opportunistic pathogens capable of causing pulmonary disease in the immunocompromised host. This subject has been reviewed extensively in previous literature.31 Other fungi, especially Candida and Aspergillus, can cause invasive pneumonia and may mimic the presentation of pulmonary mucormycosis. No serologic test is available which adequately establishes the diagnosis of any fungal pneumonia.5, 31 Candida is differentiated from Mucorales by a somewhat different clinical setting,

Management

Experience in the management of patients with pulmonary mucormycosis has been limited to date because most cases are diagnosed post-mortem. As a greater percentage of patients are diagnosed antemortem, the need for more effective therapy has become apparent.10 Control of the patient's underlying disease, although probably the most important factor, is often the most difficult objective. Withdrawal of immunosuppressive agents and corticosteroid drugs are advised when possible, as well as control

Acknowledgment

We appreciate the secretarial assistance of Sharon Udovich and Shirley Letten in the preparation of this manuscript.

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    Supported in part by the Institutional NRSA no. HL-07185 and Pulmonary Vascular SCOR no. 19155.

    Fellow in Pulmonary Disease.

    Chief Resident in Medicine.

    §

    Associate Professor of Medicine.

    Assistant Professor of Medicine and Anesthesia.

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