Bronchiolitis obliterans

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],;Associate Editor(s)-in-Chief: Hadeel Maksoud M.D.[2]

Synonyms and keywords: Obliterative bronchiolitis; proliferative bronchiolitis; bronchiolitides; popcorn lung; popcorn lung disease; popcorn workers’ lung; diacetyl-induced bronchiolitis obliterans

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Hadeel Maksoud M.D.[2]

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hereditary pancreatitis from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References

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Historical Perspective

Overview

Bronchiolitis was first described in 1822, at the university of Minnesota. Bronchiolitis obliterans was first established as a separate pathological entity and fully described by Dr. Lange, a physician, in 1901. The recent popular trend of vaping, in particular flavored vapors, has brought about an increased incidence in irreversible bronchiolitis obliterans, also known as popcorn lung disease.

Historical Perspective

Discovery

Bronchiolitis was first described in 1822, at the university of Minnesota.^[1]
Bronchiolitis obliterans was first established as a separate pathological entity and fully described by Dr. Lange, a physician, in 1901.
In 1941, LaDue was the first to discover the association between irritant fumes and the development of bronchiolitis obliterans.

Outbreaks

Bronchiolitis obliterans became prevalent amongst popcorn factory workers that inhaled diacetyl contained within the butter flavoring.

Impact on Cultural History

The recent popular trend of vaping, in particular flavored vapors, has brought about an increased incidence in irreversible bronchiolitis obliterans, also known as popcorn lung disease.

References

↑ Guerry-Force ML, Müller NL, Wright JL, Wiggs B, Coppin C, Pare PD, Hogg JC (March 1987). “A comparison of bronchiolitis obliterans with organizing pneumonia, usual interstitial pneumonia, and small airways disease”. Am. Rev. Respir. Dis. 135 (3): 705–12. doi:10.1164/arrd.1987.135.3.705. PMID 3826896.

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Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Classification

There is no consensus classification scheme for bronchiolitis subtypes. One author describes four specific subtypes as follows: cellular bronchiolitis, respiratory bronchiolitis, bronchiolitis obliterans and bronchiolitis obliterans with intraluminal polyps (proliferative bronchiolitis obliterans).

References

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Pathophysiology

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References

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Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Causes

Bronchiolitis obliterans has many possible causes, including: collagen vascular disease, transplant rejection in organ transplant patients, viral infection (RSV, adenovirus, PCP, HIV, CMV), drug reaction, aspiration and complications of prematurity (bronchopulmonary dysplasia), and exposure to toxic fumes, including: diacetyl, sulfur dioxide, nitrogen dioxide, ammonia, chlorine, thionyl chloride, methyl isocyanate, hydrogen fluoride, hydrogen bromide, hydrogen chloride, hydrogen sulfide, phosgene, polyamide-amine dyes, and ozone. Additionally, the disorder may be idiopathic (without known cause). There are many industrial inhalants that are known to cause bronchiolitis.

References

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Differentiating Bronchiolitis obliterans from other Diseases

Differential diagnosis

Bronchiolitis obliterans must be differentiated from other cavitary lung lesions.

Causes of lung cavities	Differentiating Features	Differentiating radiological findings	Diagnosis confirmation
Malignancy (Primary lung cancer)^[1]	Elderly male or female^[1] Chronic smokers Presents with a low-grade fever, absence of leukocytosis, systemic complaints weight loss, fatigue Absence of factors that predispose to gastric content aspiration, no response to antibiotics within 10 days Hemoptysis is commonly associated with bronchogenic carcinoma	A coin-shaped lesion with thick wall(>15mm) is seen on CXR with less ground glass opacities^[2] ^[3] Bronchoalveolar lavage cytology shows malignant cells	Biopsy of lung
Pulmonary Tuberculosis	Mostly in endemic areas Symptoms include productive cough, night sweats, fever, and weight loss	CXR and CT demonstrates cavities in the upper lobe of the lung	Sputum smear positive for acid-fast bacilli and nucleic acid amplification tests (NAAT) is used on sputum or any sterile fluid for rapid diagnosis and is positive for mycobacteria.
Necrotizing Pneumonia	Any age group Acute, fulminant life threating complication of prior infection >100.4 °F fever, with hemodynamic instability Worsening pneumonia-like symptoms	CXR demonstrates multiple cavitary lesions Pleural effusion and empyema are common findings	CBC is positive for causative organism
Loculated empyema	Children and elderly are at risk Pleuritic chest pain, dry cough, fever with chills Dullness to percussion decreased breath sounds, and reduced vocal resonance on examination	Empyema appears lenticular in shape and has a thin wall with smooth luminal margins	Thoracocentesis
Granulomatosis with polyangiitis (Wegener’s)^[4]	Women are more commonly effected than man^[5] Kidneys are also involved Upper respiratory tract symptoms, perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis. Lower respiratory tract symptoms (e.g. hemoptysis, cough, dyspnea) Renal symptoms, hematuria, red cell casts	Pulmonary nodules with cavities and infiltrates are a frequent manifestation on CXR	Positive for P-ANCA Biopsy of the tissue involved shows necrotizing granulomas^[4]
Rheumatoid nodule	Elderly females of 40-50 age group Manifestation of rheumatoid arthritis Presents with other systemic symptoms including symmetric arthritis of the small joints of the hands and feet with morning stiffness are common manifestations	Pulmonary nodules with cavitation are located in the upper lobe (Caplan syndrome) on X-ray	Positive for both rheumatoid factor and anticyclic citrullinated peptide antibody.
Sarcoidosis	More common in African-American females Often asymptomatic except for enlarged lymph nodes^[6] Associated with restrictive lung disease Erythema nodosum Lupus pernio (skin lesions on face resembling lupus) Bell palsy Epithelioid granulomas containing microscopic Schaumann and asteroid bodies	On CXR bilateral adenopathy and coarse reticular opacities are seen CT of the chest demonstrates extensive hilar and mediastinal adenopathy Additional findings on CT include fibrosis (honeycomb, linear, or associated with bronchial distortion), pleural thickening, and ground-glass opacities.^[7]	Biopsy of lung shows non-caseating granuloma
Bronchiolitis obliterans (Cryptogenic organizing pneumonia)^[8]^[9]	Rare condition and mimics asthma, pneumonia and emphysema It is caused by drug or toxin exposure, autoimmune diseases, viral infections, or radiation injury People working in industries are at high risk Presents with fever, cough, wheezing, and shortness of breath over weeks to months^[10]	Common appearance on CT is patchy consolidation,often accompanied by ground-glass opacities and nodules.^[11]	Biopsy of the lung^[9] Pulmonary function tests demonstrate low fev1/fvc
Langerhans cell Histiocytosis^[12]	Exclusively afflicts smokers, with a peak age of onset of between 20 and 40 years Clinical presentation varies, but symptoms generally include months of dry cough, fever, night sweats, and weight loss Skin is involved in 80% of the cases, scaly erythematous rash is typical	Thin-walled cystic cavities are the usual radiographic manifestation, observed in over 50% of patients by either CXR or CT scans.^[13]	Biopsy of the lung

References

↑ ^1.0 ^1.1 Chaudhuri MR (1973). “Primary pulmonary cavitating carcinomas”. Thorax. 28 (3): 354–66. PMC 470041. PMID 4353362.
↑ Mouroux J, Padovani B, Elkaïm D, Richelme H (1996). “Should cavitated bronchopulmonary cancers be considered a separate entity?”. Ann. Thorac. Surg. 61 (2): 530–2. doi:10.1016/0003-4975(95)00973-6. PMID 8572761.
↑ Onn A, Choe DH, Herbst RS, Correa AM, Munden RF, Truong MT, Vaporciyan AA, Isobe T, Gilcrease MZ, Marom EM (2005). “Tumor cavitation in stage I non-small cell lung cancer: epidermal growth factor receptor expression and prediction of poor outcome”. Radiology. 237 (1): 342–7. doi:10.1148/radiol.2371041650. PMID 16183941.
↑ ^4.0 ^4.1 Langford CA, Hoffman GS (1999). “Rare diseases.3: Wegener’s granulomatosis”. Thorax. 54 (7): 629–37. PMC 1745525. PMID 10377211.
↑ Lee KS, Kim TS, Fujimoto K, Moriya H, Watanabe H, Tateishi U, Ashizawa K, Johkoh T, Kim EA, Kwon OJ (2003). “Thoracic manifestation of Wegener’s granulomatosis: CT findings in 30 patients”. Eur Radiol. 13 (1): 43–51. doi:10.1007/s00330-002-1422-2. PMID 12541109.
↑ Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ, McLennan G, Moller DR, Newman LS, Rabin DL, Rose C, Rybicki B, Weinberger SE, Terrin ML, Knatterud GL, Cherniak R (2001). “Clinical characteristics of patients in a case control study of sarcoidosis”. Am. J. Respir. Crit. Care Med. 164 (10 Pt 1): 1885–9. doi:10.1164/ajrccm.164.10.2104046. PMID 11734441.
↑ Brauner MW, Grenier P, Mompoint D, Lenoir S, de Crémoux H (1989). “Pulmonary sarcoidosis: evaluation with high-resolution CT”. Radiology. 172 (2): 467–71. doi:10.1148/radiology.172.2.2748828. PMID 2748828.
↑ Murphy J, Schnyder P, Herold C, Flower C (1998). “Bronchiolitis obliterans organising pneumonia simulating bronchial carcinoma”. Eur Radiol. 8 (7): 1165–9. doi:10.1007/s003300050527. PMID 9724431.
↑ ^9.0 ^9.1 Al-Ghanem S, Al-Jahdali H, Bamefleh H, Khan AN (2008). “Bronchiolitis obliterans organizing pneumonia: pathogenesis, clinical features, imaging and therapy review”. Ann Thorac Med. 3 (2): 67–75. doi:10.4103/1817-1737.39641. PMC 2700454. PMID 19561910.
↑ Cordier JF, Loire R, Brune J (1989). “Idiopathic bronchiolitis obliterans organizing pneumonia. Definition of characteristic clinical profiles in a series of 16 patients”. Chest. 96 (5): 999–1004. PMID 2805873.
↑ Lee KS, Kullnig P, Hartman TE, Müller NL (1994). “Cryptogenic organizing pneumonia: CT findings in 43 patients”. AJR Am J Roentgenol. 162 (3): 543–6. doi:10.2214/ajr.162.3.8109493. PMID 8109493.
↑ Suri HS, Yi ES, Nowakowski GS, Vassallo R (2012). “Pulmonary langerhans cell histiocytosis”. Orphanet J Rare Dis. 7: 16. doi:10.1186/1750-1172-7-16. PMC 3342091. PMID 22429393.
↑ Moore AD, Godwin JD, Müller NL, Naidich DP, Hammar SP, Buschman DL, Takasugi JE, de Carvalho CR (1989). “Pulmonary histiocytosis X: comparison of radiographic and CT findings”. Radiology. 172 (1): 249–54. doi:10.1148/radiology.172.1.2787035. PMID 2787035.

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Epidemiology and Demographics

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References

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Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Risk Factors

Industrial workers who are at a greater risk for developing bronchiolitis obliterans:

Nylon-flock workers
Workers who spray prints onto textiles with polyamide-amine dyes
Battery workers who are exposed to thionyl chloride fumes
Workers at plants that use or manufacture flavorings, e.g. diacetyl butter-like flavoring

References

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Screening

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References

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Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Natural History, Complications and Prognosis

This disease is irreversible and severe cases often require a lung transplant. Evaluation of interventions to prevent bronchiolitis obliterans relies on early detection of abnormal spirometry results or unusual decreases in repeated measurements.

References

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Diagnosis

Treatment

Case Studies

Case #1

Related Chapters

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[pmid3826896-1] Guerry-Force ML, Müller NL, Wright JL, Wiggs B, Coppin C, Pare PD, Hogg JC (March 1987). “A comparison of bronchiolitis obliterans with organizing pneumonia, usual interstitial pneumonia, and small airways disease”. Am. Rev. Respir. Dis. 135 (3): 705–12. doi:10.1164/arrd.1987.135.3.705. PMID 3826896.

[pmid4353362-1] 1.0 ^1.1 Chaudhuri MR (1973). “Primary pulmonary cavitating carcinomas”. Thorax. 28 (3): 354–66. PMC 470041. PMID 4353362.

[pmid8572761-2] Mouroux J, Padovani B, Elkaïm D, Richelme H (1996). “Should cavitated bronchopulmonary cancers be considered a separate entity?”. Ann. Thorac. Surg. 61 (2): 530–2. doi:10.1016/0003-4975(95)00973-6. PMID 8572761.

[pmid16183941-3] Onn A, Choe DH, Herbst RS, Correa AM, Munden RF, Truong MT, Vaporciyan AA, Isobe T, Gilcrease MZ, Marom EM (2005). “Tumor cavitation in stage I non-small cell lung cancer: epidermal growth factor receptor expression and prediction of poor outcome”. Radiology. 237 (1): 342–7. doi:10.1148/radiol.2371041650. PMID 16183941.

[pmid10377211-4] 4.0 ^4.1 Langford CA, Hoffman GS (1999). “Rare diseases.3: Wegener’s granulomatosis”. Thorax. 54 (7): 629–37. PMC 1745525. PMID 10377211.

[pmid12541109-5] Lee KS, Kim TS, Fujimoto K, Moriya H, Watanabe H, Tateishi U, Ashizawa K, Johkoh T, Kim EA, Kwon OJ (2003). “Thoracic manifestation of Wegener’s granulomatosis: CT findings in 30 patients”. Eur Radiol. 13 (1): 43–51. doi:10.1007/s00330-002-1422-2. PMID 12541109.

[pmid11734441-6] Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ, McLennan G, Moller DR, Newman LS, Rabin DL, Rose C, Rybicki B, Weinberger SE, Terrin ML, Knatterud GL, Cherniak R (2001). “Clinical characteristics of patients in a case control study of sarcoidosis”. Am. J. Respir. Crit. Care Med. 164 (10 Pt 1): 1885–9. doi:10.1164/ajrccm.164.10.2104046. PMID 11734441.

[pmid2748828-7] Brauner MW, Grenier P, Mompoint D, Lenoir S, de Crémoux H (1989). “Pulmonary sarcoidosis: evaluation with high-resolution CT”. Radiology. 172 (2): 467–71. doi:10.1148/radiology.172.2.2748828. PMID 2748828.

[pmid9724431-8] Murphy J, Schnyder P, Herold C, Flower C (1998). “Bronchiolitis obliterans organising pneumonia simulating bronchial carcinoma”. Eur Radiol. 8 (7): 1165–9. doi:10.1007/s003300050527. PMID 9724431.

[pmid19561910-9] 9.0 ^9.1 Al-Ghanem S, Al-Jahdali H, Bamefleh H, Khan AN (2008). “Bronchiolitis obliterans organizing pneumonia: pathogenesis, clinical features, imaging and therapy review”. Ann Thorac Med. 3 (2): 67–75. doi:10.4103/1817-1737.39641. PMC 2700454. PMID 19561910.

[pmid2805873-10] Cordier JF, Loire R, Brune J (1989). “Idiopathic bronchiolitis obliterans organizing pneumonia. Definition of characteristic clinical profiles in a series of 16 patients”. Chest. 96 (5): 999–1004. PMID 2805873.

[pmid8109493-11] Lee KS, Kullnig P, Hartman TE, Müller NL (1994). “Cryptogenic organizing pneumonia: CT findings in 43 patients”. AJR Am J Roentgenol. 162 (3): 543–6. doi:10.2214/ajr.162.3.8109493. PMID 8109493.

[pmid22429393-12] Suri HS, Yi ES, Nowakowski GS, Vassallo R (2012). “Pulmonary langerhans cell histiocytosis”. Orphanet J Rare Dis. 7: 16. doi:10.1186/1750-1172-7-16. PMC 3342091. PMID 22429393.

[pmid2787035-13] Moore AD, Godwin JD, Müller NL, Naidich DP, Hammar SP, Buschman DL, Takasugi JE, de Carvalho CR (1989). “Pulmonary histiocytosis X: comparison of radiographic and CT findings”. Radiology. 172 (1): 249–54. doi:10.1148/radiology.172.1.2787035. PMID 2787035.

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Bronchiolitis obliterans

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hereditary pancreatitis from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References

Overview

Historical Perspective

Discovery

Outbreaks

Impact on Cultural History

References

Overview

Classification

References

References

Overview

Causes

References

Differential diagnosis

References

References

Overview

Risk Factors

References

References

Overview

Natural History, Complications and Prognosis

References

Diagnosis

Treatment

Case Studies

Related Chapters

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