Last reviewed: 25 Aug 2023
Last updated: 14 Oct 2022



History and exam

Key diagnostic factors

  • cough
  • sputum production
  • crackles, high-pitched inspiratory squeaks and rhonchi
  • dyspnea
  • fever
More key diagnostic factors

Other diagnostic factors

  • fatigue
  • hemoptysis
  • rhinosinusitis
  • weight loss
  • wheezing
  • pleuritic chest pain
  • clubbing
Other diagnostic factors

Risk factors

  • cystic fibrosis
  • host immunodeficiency
  • previous infections
  • congenital disorders of the bronchial airways
  • primary ciliary dyskinesia
  • alpha-1 antitrypsin deficiency
  • connective tissue disease
  • inflammatory bowel disease
  • aspiration or inhalation injury
  • focal bronchial obstruction
  • tall, thin, white females, age 60 or over
  • prematurity
More risk factors

Diagnostic investigations

1st investigations to order

  • high-resolution chest CT
  • CXR
  • CBC
  • sputum culture and sensitivity
  • serum alpha-1 antitrypsin phenotype and level
  • serum immunoglobulins
  • sweat chloride test
  • rheumatoid factor
  • specific IgE or skin prick test to Aspergillus fumigatus
  • serum HIV antibody
  • nasal nitric oxide (NNO)
  • pulmonary function tests
More 1st investigations to order

Investigations to consider

  • primary ciliary dyskinesia (PCD) testing
  • cystic fibrosis transmembrane regulator (CFTR) protein gene mutation testing
  • swallow study
  • pH monitoring of esophagus
  • 6-minute walk test
  • tuberculosis testing
  • diagnostic bronchoscopy with bronchoalveolar lavage
More investigations to consider

Treatment algorithm


initial presentation


acute exacerbation: mild to moderate underlying disease

acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics

3 or more exacerbations per year despite maintenance therapy


first or new isolation of Pseudomonas aeruginosa at outpatient review



Anne E. O'Donnell, MD

Professor of Medicine

Chief, Division of Pulmonary, Critical Care and Sleep Medicine

The Nehemiah and Naomi Cohen Chair in Pulmonary Disease Research

Georgetown University Medical Center




AEOD has received scientific consulting fees from the following companies: Bayer, Xellia, Horizon, Grifols, Insmed, and Electromed. AEOD is the Principal Investigator for trials sponsored by the following companies (research funding provided directly to Georgetown University): Bayer, Insmed, Aradigm, Parion, Zambon. AEOD has been a faculty member at the annual meetings of CHEST (American College of Chest Physicians), the American Thoracic Society, and the European Respiratory Society. AEOD is the author of a reference cited in this topic.


Dr Anne E. O'Donnell would like to gratefully acknowledge Dr Pamela J. McShane and Dr Sangeeta M. Bhorade, previous contributors to this topic.


PJM and SMB declare that they have no competing interests.

Peer reviewers

Philip W. Ind, BA (Cantab), MB BChir, MA (Cantab), FRCP

Emeritus Professor of Practice (Respiratory Medicine)

National Heart and Lung Institute

Imperial College London




PWI declares that he has no competing interests.

Meg Coleman, MBBS, BSc, MRCP

Respiratory Consultant

Honorary Senior Clinical Lecturer

Imperial College Hospitals NHS Trust and National Heart and Lung Institute




MC has received honoraria from Pfizer and Gilead for delivering educational sessions.

Amanda Messinger, MD, MS

Assistant Professor

Section of Pediatric Pulmonary and Sleep Medicine

Department of Pediatrics

Children's Hospital Colorado

University of Colorado Denver School of Medicine




AM declares that she has no competing interests.

Ware Kuschner, MD

Associate Professor of Medicine

Stanford University


Staff Physician

US Department of Veterans Affairs

Palo Alto Health Care System

Palo Alto



WK declares that he has no competing interests.

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