SYSTEMIC AND PARENCHYMAL LUNG DISEASES

Pneumoconiosis Key points

Chris Barber C Pneumoconiosis usually takes many years to develop and
David Fishwick affects only a proportion of similarly exposed workers

C The potential for harm depends on a range of factors including

the particle size of the dust or fibre, cumulative inhaled dose,
Abstract
clearance rates and inherent toxicity
The term ‘pneumoconiosis’ is used to describe a set of lung diseases
caused by the repeated inhalation of small particles in which long-term
C Common types of pneumoconiosis include coal worker’s
retention in the lung is a key causative factor. The site of damage
pneumoconiosis, silicosis and asbestosis
within the lung is a function of both the size and the toxicity of the
inhaled dust, fume or fibre. The ability of different types of particles
C The radiological severity of pneumoconiosis can be graded by
to cause fibrosis varies widely: crystalline silica is highly fibrogenic,
using the International Labour Organization classification
whereas iron oxide is not. In susceptible individuals, pneumoconiosis
(Table 2)
usually develops after many years of exposure, sometimes presenting
after retirement. Where cases are detected during employment (e.g.
C Treatments for pneumoconiosis are only supportive, making
during health surveillance), reduction or cessation of further exposures
early detection by health surveillance very important
should be the goal. Prevention is of prime importance as the lung dam-
age caused by pneumoconiosis is irreversible, and the retained sub-
C Where appropriate, management of existing cases should
stance may continue to cause harm many years after exposure has
focus on prevention of further exposure, smoking cessation
ceased.
and compensation advice
Keywords Coal; complicated; COPD; dust; lung; occupation;
pneumoconiosis; silica; simple

Only a proportion of similarly exposed workers develop

pneumoconiosis, suggesting that genetic factors can be relevant
Introduction in this type of disease. No specific therapies are available, so
prevention of exposure and early detection of disease are of
The term ‘pneumoconiosis’ is used to describe a set of occupa- prime importance. Selected patients should be referred for
tional lung diseases associated with inhalation of an agent (dust, consideration of transplantation.
fume, fibre) in which retention in the lung is a key causative This article focuses on the major causative exposures
factor. The term is most commonly used in the context of coal responsible for pneumoconiosis in the UK (coal, silica, asbestos).
worker’s pneumoconiosis (CWP) and silicosis, but a number of Other causes are listed in Table 1.
other agents are implicated. Pneumoconiosis generally develops
in susceptible individuals after many years of relevant industrial
exposure, and can progress after exposure has ended. Coal worker’s pneumoconiosis
The ability of different types of retained particles to cause lung
Epidemiology
damage varies widely; for example, silica dust is highly fibro-
Although the global consumption of coal as fuel has continued to
genic, whereas iron oxide dust is not. This depends on the size
rise,1 coal production in the UK has dramatically decreased over
and toxicity of the inhaled particle, as well as the lung’s ability to
the last few decades. This has been associated with a falling
clear it. Generally, particles with a median diameter of 0.5e10
mortality and incidence of CWP over the past 10e15 years (with
micrometres can penetrate into the alveoli, and those that are
approximately 140 deaths and 250e300 new cases each year).
toxic to host cells can cause permanent harm. Many mechanisms
The risk of CWP relates to the duration and level of exposure, as
are likely to be specific to the individual causative agent; in
well as the rank (carbon content) of the coal. In addition, miners
general, however, release of proinflammatory cytokines, initially
exposed to dust with high quartz content (10e15%) can develop
from alveolar macrophages, causes fibroblast formation and
a disease similar to silicosis. CWP can be simple or complicated
eventual fibrosis.
depending on the absence or presence of large opacities.

Clinical features
Chris Barber MRCP AFOM MD is a Consultant Respiratory Physician at
Sheffield Teaching Hospitals NHS Foundation Trust, and Deputy Simple coal-worker’s pneumoconiosis is associated with small
Chief Medical Officer at the Centre for Workplace Health, UK. rounded nodules predominantly in the upper zones of the lung.
Competing interests: none declared. These coal macules represent collections of dust-laden macro-
phages and are visible on a chest X-ray. In the absence of coex-
David Fishwick FRCP AFOM MD is a Consultant Respiratory Physician
and Honorary Professor of Occupational and Environmental isting chronic obstructive pulmonary disease (COPD), simple
Respiratory Medicine at the University of Sheffield and the Northern CWP is not usually associated with symptoms, physical signs or
General Hospital, Sheffield, UK. Competing interests: none declared. abnormal physiology.

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 SYSTEMIC AND PARENCHYMAL LUNG DISEASES

Other causes of pneumoconiosis

Disease Agent Notes

Non-fibrogenica
Siderosis Iron oxide Causes no symptoms, signs or physiological
abnormality
When co-exposure with silica occurs, it can
attenuate the fibrosis seen (so-called ‘mixed-
dust fibrosis’)
Stannosis Tin Marked radiological abnormality as a
consequence of high atomic number
No symptoms, signs or physiological
abnormality
Others Barium sulphate (baritosis) Marked radiological abnormality as a
consequence of high atomic number
No symptoms, signs or physiological
abnormality
Chromite, zirconium, antimony Marked radiological abnormality as a
consequence of high atomic number
No symptoms, signs or physiological
abnormality
Fibrogenic
Berylliosis Beryllium Sarcoid-like condition with fibrosis
May respond to corticosteroids
a
Not thought to cause significant pulmonary fibrosis.

Table 1

Complicated coal-worker’s pneumoconiosis usually occurs on Investigation

a background of the simple form, although it rarely occurs de Radiology: the diagnosis of simple or complicated CWP is based
novo. It is associated with slowly progressive upper lobe mass on typical radiological findings and an occupational history of
formation and fibrosis (previously termed progressive massive appropriate coal dust exposure. If available, previous radiology is
fibrosis). Complicated forms are commonly associated with particularly helpful if it shows relatively stable appearances over
cough, breathlessness and, in certain cases, progression to hyp- many years (Figure 1). Patients may also recall being told that
oxia and right heart failure. they had ‘signs of dust’ on health surveillance X-rays taken while
they were still working. Where radiological features are not
typical, particularly if there is a significant smoking history, pa-
tients with lung nodules and mass lesions should be discussed
with the lung cancer multidisciplinary teams. Particular diag-
International Labour Organization classification of small nostic difficulty may occur in Caplan’s syndrome (CWP plus
and large opacities rheumatoid arthritis), in which cavitating nodules are seen that
Small opacities mimic other conditions such as lung metastases, Wegener’s
Category granulomatosis and tuberculosis.
C 0 None (or fewer than category 1)
C 1 Small opacities, few in number Serology: in CWP, both rheumatoid factor and antinuclear
C 2 Small opacities, numerous antibody may be present in the serum, although neither is suf-
C 3 Small opacities, very numerous, plus obscure lung markings ficiently sensitive to be helpful in diagnosis.
Large opacities
Management and prevention
Category
There are no effective medical treatments for CWP, other than
C A Greatest diameter 10e50 mm
management of hypoxia and right heart failure. Coal dust is also
C B One or more opacities larger than category A, with combined
a cause of COPD, and coexisting airways obstruction should be
area less than that of the right upper lobe
treated. The importance of smoking cessation should also be
C C One or more opacities with combined area more than that of the
highlighted. Identifying simple CWP by health surveillance and
right upper lobe
reducing further exposures, in an attempt to prevent complicated
Table 2 disease, is key.

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 SYSTEMIC AND PARENCHYMAL LUNG DISEASES

of newer quartz-containing engineered stone kitchen

a
worktops.4

Clinical features and investigations

Exposure to RCS can result in a number of different disease
patterns, with differing rates of progression and prognoses.

Chronic silicosis
Chronic silicosis usually results from at least 10 years of low-
level RCS exposure: simple silicosis is usually asymptomatic
and often found on routine chest X-ray. High-resolution
computed tomography (HRCT) scanning provides additional
detail, typically demonstrating diffuse, dense, small, rounded
opacities that are predominantly seen in the upper lobes bilat-
erally. Hilar calcification (so-called ‘egg-shell’ calcification) is
also commonly seen. As with CWP, simple disease can progress
b to complicated disease, with significant respiratory disability.

Accelerated silicosis can develop after a shorter period (usually

5e10 years) of higher level RCS exposure. Progression is more
rapid than in chronic disease, the pulmonary disease being more
diffuse and the prognosis significantly worse.

Acute silicosis is associated with immediate lung damage due to

massive RCS exposure over a period of weeks or months,
commonly from hard-rock tunnelling or sand blasting. Workers
develop rapidly progressive breathless and cyanosis, with radi-
ology suggestive of pulmonary oedema. Proteinaceous and lipid-
containing material can be retrieved on bronchoalveolar lavage,
in a similar manner to primary pulmonary alveolar proteinosis.
Acute silicosis has a very poor prognosis, commonly being fatal
within a few months of presentation.

Associated conditions: silicosis is associated with a range of

other pulmonary and non-pulmonary diseases. Silica is a recog-
nized lung carcinogen, and the risk of lung cancer appears to be
increased in individuals with silicosis. Typical and atypical
Figure 1 Typical chest X-ray appearances of complicated coal mycobacterial infections are also more common, and recurrent
worker’s pneumoconiosis. Multiple small dense nodules are seen in tuberculosis in miners can be related to both reactivation and
both the upper and mid zones, with a 3-cm opacity in the right middle
reinfection (transmitted from work colleagues). Caplan’s syn-
zone (a). Radiographic appearances remained relatively stable over a
4-year follow-up period (b).
drome can occur in those with coexisting rheumatoid arthritis,
and scleroderma is more common in patients with silicosis.
Finally, various data are supportive of a causative link between
Silicosis RCS and COPD, even in the absence of radiological evidence of
silicosis.
Epidemiology
The incidence and mortality of silicosis have been relatively
Management and prevention
stable in Britain over the last decade, with approximately 10
As with CWP, there is no specific treatment for silicosis, so
deaths and 40e45 new cases assessed for industrial benefits per
prevention or early detection of disease is vital. Therapeutic
year. This is in marked contrast to the situation in certain
whole-lung lavage has been undertaken in the acute form, but
developing countries where pneumoconiosis is the most com-
the prognosis remains very poor. Complications such as right
mon form of occupational lung disease, and the incidence of
heart failure, coexisting airways disease and mycobacterial
silicosis is rising.2
infection should be managed conventionally. In addition,
Various occupations are associated with exposure to respi-
smoking cessation advice is important.
rable crystalline silica (RCS), most commonly from rock, stone
or sand exposure. These include stone masonry, foundry work,
Asbestosis
rock tunnelling, quarry working, mining, refractory brick
manufacture and construction work. In addition, outbreaks of Epidemiology
disease have occurred in new industries, where high-level sil- ‘Asbestosis’ is the term used to describe diffuse pulmonary
ica exposure has occurred in denim sandblasters3 and installers fibrosis caused by the inhalation of asbestos fibres. Unlike other

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 SYSTEMIC AND PARENCHYMAL LUNG DISEASES

types of pneumoconiosis, mortality from asbestosis in Britain is pulmonary fibrosis, showing restriction and reduced transfer
rising, with around 450 deaths and 900 new benefit applications factor.
each year. These represent the historical legacy of extensive in-
dustrial use of asbestos in Britain, as well as a disease with very Management
long latency. Asbestos use has been banned in many developed Asbestosis typically progresses relatively slowly. There are no
countries, but occupational exposure can still occur from dis- specific treatments other than treatment of right heart failure and
turbing asbestos-containing materials in older buildings. In other smoking cessation advice. Current anti-fibrotic agents (e.g. pir-
countries, asbestos is still produced and used commercially. fenidone, nintedanib) that are available for use in mild to mod-
Globally in 2014, around 2 million metric tonnes of asbestos erate IPF have not been licensed for asbestosis.
were mined, with China, India, Russia and Brazil being the
biggest users. Male mortality rates from asbestosis can be pre- Compensation
dicted from consideration of historic per capita asbestos usage, Compensation and other benefits may be available in certain
with due consideration of latency.5 countries where individuals have been harmed by the devel-
opment of pneumoconiosis as a result of occupational
Clinical features exposures. A
Asbestosis usually occurs in susceptible individuals several de-
cades (usually at least 10e20 years) after prolonged occupational
exposure to asbestos. Individuals at increased risk include KEY REFERENCES
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Please cite this article in press as: Barber C, Fishwick D, Pneumoconiosis, Medicine (2016), http://dx.doi.org/10.1016/j.mpmed.2016.03.001