eMedicine Specialties > Pulmonology > Infectious Lung Diseases

Pneumonia, Fungal

Author: Romeo A Mandanas, MD, FACP, Director, Western Oklahoma Blood and Marrow Transplant Program, Site Research Leader, Cancer Care Associates-Oklahoma City
Contributor Information and Disclosures

Updated: Sep 17, 2009

Introduction

Background

Fungal pneumonia is an infectious process in the lungs caused by any one or a combination of endemic or opportunistic fungi. Endemic fungal pathogens (eg, Histoplasma capsulatum, Coccidioides immitis, Blastomyces dermatitidis, Paracoccidioides brasiliensis) cause infection in both healthy and immunocompromised hosts in defined geographic locations of the Americas and around the world. Opportunistic fungal organisms (eg, Candida species, Aspergillus species, Mucor species, Cryptococcus neoformans) tend to cause pneumonia in patients with congenital or acquired defects in their host defenses.

Pathophysiology

Infection occurs following the inhalation of spores, after the inhalation of conidia, or by the reactivation of a latent infection. Hematogenous dissemination frequently occurs, especially in an immunocompromised host.

Frequency

United States

The endemic fungi are prevalent in the Mississippi RiverValley and the Ohio River Valley (eg, H capsulatum, B dermatitidis), the southwestern United States, and northwestern Mexico (eg, C immitis).

International

These fungi have caused several pneumonia outbreaks in Argentina and other areas of Central and South America. P brasiliensis is restricted to Central and South America. African histoplasmosis, which is caused by Histoplasma capsulatum duboisii, is limited to equatorial Africa between 20° N and 10° S, which includes Gabon, Uganda, and Kenya.

The other opportunistic organisms are ubiquitous, are usually found worldwide, and tend to cause disease in hosts with abnormal immune defenses. For instance, C neoformans can affect people with intact immune systems at a rate of 0.2 cases per million population per year. Approximately 80-90% of patients with AIDS develop cryptococcosis. The advent of highly active antiretroviral therapy (HAART) has significantly reduced this incidence.1

Mortality/Morbidity

The endemic fungal pneumonias are generally self-limited in healthy hosts. C immitis is the most virulent; yet, 90% of patients recover without treatment. Patients with fungal pneumonias may develop chronic pulmonary (eg, cavitation, pleural effusions, bronchopleural fistulas) or extrapulmonary complications. However, in patients with AIDS, the mortality rate is as high as 70%.

Aspergillosis in patients who are neutropenic (from either leukemia chemotherapy or bone marrow transplantation) has a mortality rate of 50-85%. More often, the cause of mortality in patients who are immunocompromised is disseminated disease in the case of aspergillosis and candidal infections or meningoencephalitis in the case of cryptococcosis.

Race

No racial predilection is described, although C immitis causes more severe disease in patients of African American or Philippine descent.

Sex

Endemic fungal disease affects men (75-95%) more often than women; estrogen-mediated inhibition of mycelium-to-yeast transformation may be responsible for the male predominance. Estrogen seems to have a protective effect against cryptococcal infection. Cryptococcosis has a male-to-female ratio of 2-3:1.

Clinical

History

  • Fever: In individuals who are neutropenic or immunocompromised, persistent fever (even before pulmonary findings) may be an early sign of infection, especially if the fever is unresponsive to broad-spectrum antibiotics.
  • Cough, usually nonproductive
  • Pleuritic chest pain or dull discomfort
  • Dyspnea leading to respiratory failure
  • Obstructive symptoms from enlarged mediastinal adenopathy in the endemic mycoses
  • Hemoptysis in invasive aspergillosis or mucormycosis
  • History of travel to or exposure in areas containing endemic mycoses
  • Symptoms from involvement of extrapulmonary systems (may suggest disease)
  • Rheumatologic syndromes (common among endemic mycoses)
    • Arthritis and arthralgia
    • Erythema nodosum
    • Erythema multiforme
    • Pericarditis
  • Endemic mycoses  with associated dissemination
    • Skin (eg, papules, pustules, plaques, ulcers, abscesses, proliferative lesions) - May mimic skin cancer as in B dermatitidis infection
    • Bone and joints
    • Brain and meninges - Meningitis with poor prognosis (10-20%)
    • Septicemia or sepsis syndrome
  • Hypersensitivity or allergic reactions
    • Allergic bronchial asthma (Aspergillus species, Candida species)
    • Allergic bronchopulmonary mycoses (Aspergillus species, Candida species)
    • Bronchocentric granulomatosis (necrotizing granulomatous replacement and eosinophilic infiltration of bronchial mucosa in infection with Aspergillus species)
    • Extrinsic allergic alveolitis (malt worker's lung, farmer's lung)
  • Extrapulmonary sites in individuals who are immunocompromised
  • Meningoencephalitis in patients with AIDS and cryptococcosis
  • Skin (often a good site for biopsy)
  • CNS (brain abscess in infection with Aspergillus and Mucor species)
  • Kidneys
  • Liver and spleen (hepatosplenic candidiasis)
  • Muscle (Candida species)
  • Eye (endophthalmitis) in Candida species infection
  • Nasal passages and sinuses (Aspergillus and Mucor species)
  • Bloodstream and bone marrow (sepsis syndrome)

Physical

  • Temperature elevation and tachycardia
  • Respiratory distress, rales, signs of pulmonary consolidation, and pleural rub
  • Important possible extrapulmonary findings
    • Meningitis (neck stiffness, headaches, mental status change)
    • Skin lesions (pustules, papules, plaques, nodules, ulcers, abscesses, hemorrhagic lesions)
    • Nasal passage and sinuses (Mucor and Aspergillus species)
    • Rheumatologic and allergic findings

Causes

  • Workers or farmers with heavy exposure to bird, bat, or rodent droppings and other animal excreta in endemic areas are predisposed to acquire any of the endemic fungal pneumonias.
  • C immitis, because of its virulence, is also a threat among laboratory personnel working with this fungus.
  • Conditions that predispose patients to any of the opportunistic fungal pathogens are as follows:
    • Acute leukemia or lymphoma during myeloablative chemotherapy
    • Bone marrow or Peripheral Blood Stem Cell transplantation
    • Solid organ transplantation on immunosuppressive treatment
    • Prolonged corticosteroid therapy
    • Acquired immunodeficiency syndrome
    • Prolonged neutropenia from various causes
    • Congenital immune deficiency syndromes
    • Postsplenectomy state
    • Genetic predisposition: Certain toll-like receptor (TLR) polymorphisms (eg, TLR 4 haplotype S4) in the unrelated donor can increase the risk of invasive aspergillosis in the recipient of a stem cell transplant from a matched but unrelated donor.2 Similarly, TLR1 and TLR6 polymorphisms in the recipient have been associated with susceptibility to invasive aspergillosis after allogeneic stem cell transplantation.3

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References

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Further Reading

Keywords

fungal pneumonia, pulmonary mycosis, mycotic pneumonia, , endemic fungal pneumonia, aspergillosis, meningoencephalitis, cryptococcosis, acute leukemia, lymphoma, myeloablative chemotherapy, bone marrow transplantation, peripheral blood stem cell transplantation, allogeneic stem cell transplantation, unrelated donor transplantation, graft-versus-host disease, graft versus host disease, solid organ transplantation on immunosuppressive treatment, prolonged corticosteroid therapy, AIDS, congenital immune deficiency syndromes

Contributor Information and Disclosures

Author

Romeo A Mandanas, MD, FACP, Director, Western Oklahoma Blood and Marrow Transplant Program, Site Research Leader, Cancer Care Associates-Oklahoma City
Romeo A Mandanas, MD, FACP is a member of the following medical societies: American College of Physicians, American Medical Association, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Oklahoma State Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Ryland P Byrd Jr, MD, Professor, Department of Internal Medicine, Division of Pulmonary Medicine and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University; Chief of Pulmonary Medicine, Medical Director of Respiratory Therapy, Intensive Care Unit, Program Director of Pulmonary Diseases and Critical Care Medicine Fellowship, James H Quillen Veterans Affairs Medical Center
Ryland P Byrd Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H, Professor, Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Southern California Keck School of Medicine
Om Prakash Sharma, MD, FRCP, FCCP, DTM&H is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Osler Society, American Thoracic Society, New York Academy of Medicine, and Royal Society of Medicine
Disclosure: Keck School of Medicine, USC None None

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
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