Hypersensitivity pneumonitis

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(Redirected from
Extrinsic allergic alveolitis
)
For the dental condition sometimes called alveolitis, see
dry socket
.
Hypersensitivity Pneumonitis
Other namesAllergic alveolitis, bagpipe lung, extrinsic allergic alveolitis (EAA)
Idiopathic Pulmonary Fibrosis, Occupational asthma, Sarcoidosis
PreventionAvoidance of antigen exposure
TreatmentAvoidance of antigen exposure and sometimes steroids

Hypersensitivity pneumonitis (HP) or extrinsic allergic alveolitis (EAA) is a syndrome caused by the repetitive inhalation of antigens from the environment in susceptible or sensitized people.[1][2] Common antigens include molds, bacteria, bird droppings, bird feathers, agricultural dusts, bioaerosols and chemicals from paints or plastics.[3] People affected by this type of lung inflammation (pneumonitis) are commonly exposed to the antigens by their occupations, hobbies, the environment and animals.[4][3] The inhaled antigens produce a hypersensitivity immune reaction causing inflammation of the airspaces (alveoli) and small airways (bronchioles) within the lung.[4] Hypersensitivity pneumonitis may eventually lead to interstitial lung disease.[5]

Signs and symptoms

Hypersensitivity pneumonitis (HP) can be categorized as acute, subacute, and chronic based on the duration of the illness.[6][3]

Acute

In the

swelling and headache. Symptoms resolve within 12 hours to several days upon cessation of exposure.[1]

Subacute

Patients with

fatigue, anorexia, weight loss, and pleurisy
. Symptoms are similar to the acute form of the disease, but are less severe and last longer. Findings may be present in patients who have experienced repeated acute attacks.

Chronic

In chronic HP, dose of the antigen tends to be low volume but for a longer duration.[2] Patients often lack a history of acute episodes. They have an insidious onset of cough, progressive dyspnea, fatigue, and weight loss. This is associated with partial to complete but gradual reversibility. Avoiding any further exposure is recommended. Clubbing is observed in 50% of patients. Tachypnea, respiratory distress, and inspiratory crackles over lower lung fields often are present.[1] In fact, hypersensitivity pneumonitis can often resemble IPF in terms of pulmonary fibrosis in that many patients experience hypoxemia.[3]

Epidemiology

Although the prevalence of hypersensitivity pneumonitis is not established it is thought to be low.[7] Data collection limitations are a result of difficulty in diagnosis, sub-clinical presentations that go undetected and variability in climate, region and proximity to local industries.[7] The most common types are bird fancier's and farmer's lung.[8][7] Interestingly, cigarette smoking appears to be protective against the disease.[3]

Pathophysiology

Hypersensitivity pneumonitis is caused by an exaggerated

immune response (hypersensitivity). Type III hypersensitivity and type IV hypersensitivity can both occur depending on the cause.[9][10] In general, acute HP is suspected to be attributed to a type III hypersensitivity while the subacute and chronic types are suspected to be caused by T cell infiltration and granuloma formation.[11] Because different people react variably to antigen exposure, the exact mechanism is unclear but genetic and host factors are likely at play.[2] The two hit hypothesis is often toted in the literature to explain why some people have a normal reaction to an antigenic exposure without clinical findings while others experience an exaggerated immune response. The "first hit" in the hypothesis is proposed to be genetic susceptibility and surrounding environmental factors and the "second hit" would be the introduction of the antigen into the respiratory system which causes the exaggerated immune response.[8]

Diagnosis

The diagnosis is made through clinical judgement using a combination of findings because there does not exist a single, universal diagnostic criteria for the disease.[12][3] The diagnosis is most commonly ascertained first with a detailed exposure history followed by a battery of clinical tests including: imaging, histopathology, pulmonary function testing, serology, bronchoscopy, and more.[3][12] In 2020, official guidelines were published by American Thoracic Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax which provides a systematic approach to the diagnosis of HP that relies on high-resolution computed tomography.[12]

Exposure History

A detailed occupational, home and environmental exposure history is the first step in diagnosis. Unfortunately, only 60% of inciting antigens are identified in exposure assessment.[12] Re-exposure to the antigen can help aid in diagnosis.[3] Standardized questionnaires have been created to help in obtaining an exposure history although no official questionnaire has been purported.[13] It has been recommended that the questionnaire administered should be relevant to the region in which the exposure has potentially occurred.[14]

Detailed exposure assessments are warranted in the cause of damp indoor environments which have the potential to propagate mold throughout the dwelling. The decision to enlist an

industrial hygienist should be made based on the answers to evidence based questions during the environmental assessment.[14] The industrial hygienist or environmental scientist will make the decision to conduct additional sampling.[14]

Types

Hypersensitivity pneumonitis may also be called many different names, based on the provoking antigen. These include:

Type[15] Specific antigen Exposure
Bird fancier's lung
Also called bird breeder's lung, pigeon breeder's lung, and poultry worker's lung 		Avian proteins Feathers and bird droppings [16]
Bagassosis
Exposure to moldy molasses 		Thermophilic actinomycetes[16] Moldy bagasse (pressed sugarcane)
Cephalosporium HP
Cephalosporium
 		Contaminated basements (from sewage)
Cheese-washer's lung Penicillium casei[16] or P. roqueforti Cheese casings
Chemical worker's lung – Isocyanate HP
Methylene bisphenyl isocyanate
(MDI) 		Paints, resins, and polyurethane foams
Chemical worker's lung[16] – Trimellitic anhydride (TMA) HP Trimellitic anhydride[16] Plastics, resins, and paints
Coffee worker's lung Coffee bean protein Coffee bean dust
Compost lung Aspergillus Compost
Detergent worker's disease Bacillus subtilis enzymes Detergent
Farmer's lung The
molds

The bacteria

Moldy hay
Hot tub lung
Mycobacterium avium
 complex 		Mist from hot tubs
Humidifier lung The bacteria

The fungi

The amoebae

Mist generated by a machine from standing water
Japanese summer house HP Also called Japanese summer-type HP
Trichosporon cutaneum
 		Damp wood and mats
Laboratory worker's lung Male rat urine protein Laboratory rats
Lycoperdonosis Puffball spores Spore dust from mature puffballs[17]
Malt worker's lung Aspergillus clavatus[16] Moldy barley
Maple bark disease Cryptostroma corticale[16] Moldy maple bark
Metalworking fluids HP Nontuberculous mycobacteria Mist from metalworking fluids
Miller's lung
Sitophilus granarius (wheat weevil)[16]
 Dust-contaminated grain[16]
Mollusc shell HP Aquatic animal proteins Mollusc shell dust
Mushroom worker's lung Thermophilic actinomycetes Mushroom compost
Peat moss worker's lung Caused by
Penicillium citreonigrum
 		Peat moss
Pituitary snuff taker's lung Pituitary snuff Medication (Diabetes insipidus)
Sauna worker's lung Aureobasidium, Graphium spp Contaminated sauna water
Sequoiosis Aureobasidium, Graphium spp Redwood bark, sawdust
Streptomyces HP Streptomyces albus Contaminated fertilizer
Suberosis Penicillium glabrum (formerly known as Penicillium frequentans) Moldy cork dust
Tap water HP Unknown Contaminated tap water
Thatched roof disease Saccharomonospora viridis Dried grass
Tobacco worker's lung Aspergillus spp Moldy tobacco
Trombone Player's lung (Brass Player's Lung)
Mycobacterium chelonae
 		Various Mycobacteria inside instruments[18][19]
Wine-grower's lung Botrytis cinerea mold Moldy grapes
Woodworker's lung Alternaria, Penicillium spp Wood pulp, dust

Of these types, Farmer's Lung and Bird-Breeder's Lung are the most common. "Studies document 8-540 cases per 100,000 persons per year for farmers and 6000-21,000 cases per 100,000 persons per year for pigeon breeders. High attack rates are documented in sporadic outbreaks. Prevalence varies by region, climate, and farming practices. HP affects 0.4–7% of the farming population. Reported prevalence among bird fanciers is estimated to be 20-20,000 cases per 100,000 persons at risk."[1]

Imaging

No single imaging finding is singularly definitive of a diagnosis rather clinicians rely on a constellation of findings. Both chest radiographs and high resolution CT scans can be normal.[1][12]

Chest Radiographs

Acute presentation may reveal poorly defined a micro-nodular interstitial pattern and ground-glass opacities in the lower and mid lung zones. In addition to this, subacute presentations may show reticular nodular opacities in mid-to-lower lung zones.[1] Chronic forms may show fibrotic changes and appear like Idiopathic pulmonary fibrosis.[3]

High-Resolution Computed Tomography

This has become a common diagnostic imaging for the diagnosis and is the modality used in the Official ATS/JRS/ALAT Clinical Practice Guideline.

traction bronchiectasis, and honeycombing, patchy ground-glass attenuation, centrilobular nodules, and mosaic attenuation.[12][20] Three-density pattern (head cheese sign) is radiological sign which shows a region of the lung with three or more different types of attenuation which can be typical for the fibrotic type.[12]

Histopathology

The acute form can be characterized by poorly formed noncaseating interstitial

mononuclear cell infiltration in a peribronchial distribution with prominent giant cells.[1] The subacute, or intermittent, form produces more well-formed noncaseating granulomas, bronchiolitis with or without organizing pneumonia, and interstitial fibrosis.[1] Much like the pathogenesis of idiopathic pulmonary fibrosis (IPF), chronic HP is related to increased expression of Fas antigen and Fas ligand, leading to increased epithelial apoptosis activation in the alveoli.[21] Cholesterol clefts or asteroid bodies are present within or outside granulomas.[1]

Pulmonary Function Testing

Pulmonary function tests (PFTs) can generally reveal a restrictive pattern

DLCO
).

Bronchoscopy

Bronchoalveolar lavage (BAL) is a reliable way to detect inflammation in the lung airways. Fluid analysis from the lavage extracted from the airways on bronchoscopy often reveals a total elevation in cell count in addition to an elevation in the percentage of T lymphocytes. This is a good way to help exclude other similar lung diseases like sarcoidosis, infection and Idiopathic pulmonary fibrosis.[8]

Lung biopsy

interstitium
is expanded by a chronic inflammatory infiltrate. Two multinucleated giant cells can be seen within the interstitium at left, and a plug of organizing pneumonia at bottom left.

Lung

interstitium by lymphocytes accompanied by an occasional multinucleated giant cell or loose granuloma.[7][22]

When fibrosis develops in chronic hypersensitivity pneumonitis, the differential diagnosis in lung biopsies includes the idiopathic interstitial pneumonias.[23] This group of diseases includes usual interstitial pneumonia, non-specific interstitial pneumonia and cryptogenic organizing pneumonia, among others.[7][22]

The prognosis of some idiopathic interstitial pneumonias, e.g. idiopathic usual interstitial pneumonia (i.e. idiopathic pulmonary fibrosis), are very poor and the treatments of little help. This contrasts the prognosis (and treatment) for hypersensitivity pneumonitis, which is generally fairly good if the allergen is identified and exposures to it significantly reduced or eliminated. Thus, a lung biopsy, in some cases, may make a decisive difference.

Serum Precipitins

Assays for serum IgGs can aid in identifying possible antigenic exposures and are used as markers of exposure [12][10] However, there use is limited in making a definitive diagnosis because serum antibody levels are often elevated in those people who are exposed to an antigen but do not have the disease.[12][3][1] Up to 90% of people exposed to the antigen have precipitins but only 50% of similarly exposed people who are asymptomatic have the same precipitins.[10] False negatives are often common with serum precipitins because of lack of testing reagents for many antigens.[3]

Precipitating IgG antibodies against fungal or avian antigens can be detected in the laboratory using the traditional Ouchterlony immunodiffusion method wherein 'precipitin' lines form on agar plate. However, the time-consuming and labor-intensive precipitin method has largely been replaced by automated IgG antibody tests. These tests can detect IgG antibodies against a variety of potential triggers including Aspergillus fumigatus (Farmer's lung or ABPA) or avian antigens (Bird Fancier's Lung). They are routinely performed on automated immunoassay systems such as ImmunoCAP or IMMULITE.[24][25]

Differential diagnosis

Organic dust toxic syndrome presents similarly with fevers, chills a few hours after exposure to bioaerosols with toxins from fungi, however this is not a true hypersensitivity reaction because it occurs on initial exposure without a preceding sensitization [1]

In chronic disease, HP must be differentiated from very similarly presenting idiopathic pulmonary fibrosis.[12]

Although overlapping in many cases, hypersensitivity pneumonitis may be distinguished from

bronchi.[11]

Similarly, sarcoidosis has noncaseating granuloma formation, however hilar adenopathy is often seen on chest radiographs.[12]

Treatment

The best treatment is to avoid the provoking allergen, as chronic exposure can cause permanent damage and acute disease is often self-limiting. The identification of the provoking antigen and its location must be ascertained by conducting an exposure assessment. Home cleaning is one method of antigen avoidance.

symptoms but may produce side-effects.[28] In the case of severe, end-stage pulmonary fibrosis arising from chronic exposure, lung transplant may be the only viable option.[3] In addition to steroids for fibrotic disease, other immunosuppressants (Azathioprine, Mycophenolic acid) and anti-fibrotic agents (Nintedanib) may be used although their effectiveness is unclear[2]

Prognosis

There are few studies examining longitudinal outcomes in patients diagnosed with hypersensitivity pneumonitis. One study in the US showed about a 0.09 to 0.29 per million increase in mortality rates although the cause specific cause was unclear.

Interstitial pulmonary fibrosis.[8]

Additional images

  • High magnification micrograph of hypersensitivity pneumonitis showing granulomatous inflammation. Trichrome stain.
    High magnification
    Trichrome stain
    .

See also

References

  1. ^ a b c d e f g h i j k l "Hypersensitivity Pneumonitis". EMedicine. June 2006.
  2. ^
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  3. ^
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  5. S2CID 13460021
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  6. ^ "Hypersensitivity Pneumonitis: Signs and Symptoms". The Regents of The University of California.
  7. ^ a b c d e Mukhopadhyay, Sanjay. "Pathology of Hypersensitivity Pneumonitis", Retrieved on 3 May 2013.
  8. ^
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  9. S2CID 31344045
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  16. ^ a b c d e f g h i j k l Kumar 2007, Table 13-5
  17. doi:10.1016/S0196-4399(97)89413-5
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  18. PMID 20823006
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  19. ^ "Sour Note: Sax Can Cause Lung Disease". ABC News. 7 September 2010. Retrieved 4 April 2018.
  20. doi:10.53347/rid-150222 (inactive 2024-04-04). Archived from the original on 2022-12-01. Retrieved 2022-12-01.{{cite journal}}: CS1 maint: DOI inactive as of April 2024 (link
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  24. doi:10.4103/0972-6691.112555
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  25. ^ Richardson MD, & Page ID (2017). Aspergillus serology: Have we arrived yet? Medical Mycology, 55(1), 48–55. https://doi.org/10.1093/mmy/myw116
  26. ^ "Lecture 14: Hypersensitivity". Archived from the original on 2006-02-06. Retrieved 2008-09-18.
  27. ^ "Allergy & Asthma Disease Management Center: Ask the Expert". Archived from the original on 2007-02-16. Retrieved 2008-09-18.
  28. ^ "Hypersensitivity Pneumonitis Treatment - Conditions & Treatments - UCSF Medical Center". www.ucsfhealth.org. Retrieved 4 April 2018.

External links

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