Cough and Nasal Pain

Putting Together an Uncommon Diagnosis

Eric A. Jensen, Prasad M. Panse, Kristopher W. Cummings, Clinton E. Jokerst, Michael Gotway

Research output: Contribution to journalArticle

Abstract

The pulmonary vasculitides include a wide spectrum of disorders that have in common inflammation and destruction of pulmonary blood vessels. Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is the most common of the antineutrophil cytoplasmic antibody-associated small-vessel vasculitides, and affects a number of organ systems. GPA affects a wide age range, although it typically presents in middle adulthood. The classic triad of GPA-upper airway involvement (sinusitis, otitis media, mucosal ulcerations, airway inflammation), lower respiratory tract disease, and glomerulonephritis-is not always evident at presentation. Furthermore, limited forms of GPA occur, in which the expected upper airway and renal involvement may be lacking. The imaging manifestations of GPA commonly include multiple, bilateral nodules or masses, frequently with cavitation or necrosis. Diffuse lung opacity due to alveolar hemorrhage, or tracheobronchial inflammation that may result in stenosis, may be encountered. Systemic GPA may be diagnosed when cytoplasmic antineutrophil cytoplasmic antibody/anti-proteinase 3 antibodies are detected in the proper clinical context, but, often, the diagnosis of GPA relies on recognition of a combination of common clinical, laboratory, and imaging findings with typical histopathologic changes noted from a biopsy of the affected tissue. A combination of a corticosteroid and immunomodulatory agents is used to induce and maintain remission in patients with GPA. Although potentially fatal if untreated, immunosuppressive therapy has dramatically improved survivorship in patients with GPA.

Original languageEnglish (US)
Pages (from-to)63-67
Number of pages5
JournalClinical Pulmonary Medicine
Volume26
Issue number2
DOIs
StatePublished - Mar 1 2019

Fingerprint

Granulomatosis with Polyangiitis
Nose
Cough
Pain
Antineutrophil Cytoplasmic Antibodies
Vasculitis
Myeloblastin
Inflammation
Respiratory Tract Diseases
Lung
Sinusitis
Otitis Media
Immunosuppressive Agents
Glomerulonephritis
Blood Vessels
Pneumonia
Adrenal Cortex Hormones
Pathologic Constriction
Necrosis
Survival Rate

Keywords

  • CT
  • cytoplasmic antineutrophil cytoplasmic antibody
  • granulomatosis with polyangiitis
  • tracheitis
  • Wegener

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

Cite this

Cough and Nasal Pain : Putting Together an Uncommon Diagnosis. / Jensen, Eric A.; Panse, Prasad M.; Cummings, Kristopher W.; Jokerst, Clinton E.; Gotway, Michael.

In: Clinical Pulmonary Medicine, Vol. 26, No. 2, 01.03.2019, p. 63-67.

Research output: Contribution to journalArticle

Jensen, Eric A. ; Panse, Prasad M. ; Cummings, Kristopher W. ; Jokerst, Clinton E. ; Gotway, Michael. / Cough and Nasal Pain : Putting Together an Uncommon Diagnosis. In: Clinical Pulmonary Medicine. 2019 ; Vol. 26, No. 2. pp. 63-67.
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