Occupational Medicine 2018;68:482–484

Advance Access publication 25 July 2018 doi:10.1093/occmed/kqy106

CASE REPORT

A case of accelerated silicosis

M. Nakládalová1, L. Štěpánek1, V. Kolek2, M. Žurková2 and T. Tichý3
1
Department of Occupational Medicine, University Hospital Olomouc and Faculty of Medicine and Dentistry, Palacký University

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Olomouc, 77900 Olomouc, Czech Republic, 2Department of Respiratory Medicine, University Hospital Olomouc and Faculty of
Medicine and Dentistry, Palacký University Olomouc, 77900 Olomouc, Czech Republic, 3Department of Clinical and Molecular
Pathology, University Hospital Olomouc and Faculty of Medicine and Dentistry, Palacký University Olomouc, 77515 Olomouc,
Czech Republic
Correspondence to: L. Štěpánek, Department of Occupational Medicine, University Hospital Olomouc and Faculty of Medicine
and Dentistry, Palacký University Olomouc, I. P. Pavlova 6, 779 00 Olomouc, Czech Republic. Tel: +420 608757316; e-mail:
stepanek.ladislav@gmail.com

Abstract Silicosis, caused by inhaling dust containing free crystalline silica, typically has a chronic course, with
the numbers of silicosis patients declining globally. Much rarer are the acute and subacute forms.
Presented is a case of severe subacute (accelerated) silicosis. The condition resulted from ~2 years
of very intense exposure without appropriate personal protective equipment while sandblasting. The
patient’s initial symptoms were progressive cough, dyspnoea and weight loss. Given his occupational
history, typical clinical manifestations and radiological findings, an initial diagnosis of accelerated
silicosis was proposed and histologically confirmed. The patient was a candidate for lung transplant-
ation. The case demonstrates a rare but largely preventable disease with serious health effects and a
poor prognosis.
Key words Accelerated silicosis; crystalline silica; lung transplantation; pneumoconiosis.

Introduction
Silicosis is most commonly a chronic condition. However,
rarely the course may be subacute (accelerated) or acute
[1]. These have been described as single case reports or
small case series in the literature. We have previously
reported a case of a sandblaster with silicosis who died
during lung transplantation (LT) <5 years from diagno-
sis and 10 years after the initial exposure [2].
While acute silicosis develops within only a few years
after the usually short but intense exposure, accelerated
silicosis (AS) mostly occurs 5–10  years from the initial
exposure to dust with high concentrations of silica par-
ticles [3]. AS both resembles acute silicosis, by accumu-
lation of granular lipoproteinaceous material in alveolar
spaces, and has features of chronic silicosis with nodules
and a more rapid progression [1,3].
Case report
In early 2017, a 28-year-old male with interstitial lung
disease and a 2-year history of sandblasting was referred
to a department of occupational medicine. The patient
who had smoked 20 cigarettes a day since being a teen-
ager reported progressive exertional dyspnoea, morning
cough and weight loss (10 kg over the previous month)
with lack of appetite. His job was to repair old car bodies
by pressure blasting with silica sand (98% SiO2) in a sheet
metal container. The only personal protective equipment
he used was a balaclava helmet. There was no dust extrac-
tion system. Based on his contract with the employer, he
only worked informally from early 2012 to early 2014.
During the initial examination, an anteroposterior
chest radiograph showed diffuse, bilateral, nearly sym-
metrically confluent, ill-defined opacities with honey-
comb emphysema. The diaphragm was slightly blurred,
with adhesions and the costophrenic angles slightly
blunted by pleural fluid (Figure 1A). A high-resolution
computed tomography scan of the lungs showed large,
almost symmetrical consolidations, particularly in the
upper and middle lung fields.
Initial spirometry and body plethysmography showed
a combined respiratory defect: forced vital capacity
(FVC) 2940 ml (55% predicted); forced expiratory vol-
ume in the first second (FEV1) 1860 ml (41%); total lung
capacity 5020 ml (68%); residual volume (RV) 1860 ml
(106%); FEV1/FVC ratio 63%. Moderately reduced car-
bon monoxide diffusing capacity (DLCO) was detected
(55% predicted).
Laboratory tests showed positive results for antineu-
trophil cytoplasmic antibodies (ANCAs), borderline
positive results for antinuclear antibodies (ANAs), mild

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 M. NAKLÁDALOVÁ ET AL.: A CASE OF ACCELERATED SILICOSIS  483
leucocytosis (13.34 × 109/L), elevated C-reactive protein
(8.7 mg/L), soluble interleukin-2 receptor (1349 kU/L),
IgA (4.77  g/L), IgG (17.2  g/L), circulating immune
complexes (CICs, 82 U) and lactate dehydrogenase
(12.32  µkat/L). Rheumatological examination showed
no evidence of systemic disease.
The other biochemical tests were normal. Further
laboratory tests detected elevated tumour markers, namely
cancer antigen (CA)-125 (70.1  µg/L) and cytokeratin
fragment 19 (CYFRA 21-1, 4.26 µg/L), neuron-specific
enolase (NSE, 46.21 µg/L) and neopterin (3.99 µg/L).
Positron emission tomography/computed tomography
demonstrated glucose hypermetabolism in irregular con-
densations in both lungs and mediastinal lymph nodes,
suggesting inflammation (Figure 1B).
Figure 1.  (A) Chest radiograph—bilateral, nearly symmetrically con-
fluent, ill-defined opacities. (B) Chest positron emission tomography/
computed tomography—glucose hypermetabolism in irregular con-
densations in both lungs and mediastinal lymph nodes.
Bronchoscopy revealed diffuse mucosal hyperaemia
with disperse flaky mucus. Cytological analysis of bron-
choalveolar lavage fluid (BALF) showed mixed, lym-
phocytic and neutrophilic, alveolitis with demonstrated
erythrocytes, multinucleated cells and pigment-laden
macrophages.
Lung tissue biopsy showed fibrosing histiocytic infil-
trates (Figure 2A) with a tiny hyaline silicotic nodule and
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secondary alveolar lipoproteinosis with cholesterol crys-
tals. Pleural fibrosis was also apparent. Polarized light
microscopy showed optically active silica crystals in both
the interstitium and the alveolar lumen (Figure 2B). The
findings were suggestive of pneumoconiosis correspond-
ing to AS.
The disease has clinically progressed since diagno-
sis. The patient’s condition was complicated by acute
bronchitis with haemoptysis. Twelve months after initial
spirometry, his respiratory parameters generally deteri-
orated: FVC 2610  ml (49% predicted); FEV1 1450  ml
(32%); DLCO 31%; RV 2740  ml (156%). The patient
was a candidate for LT and has been placed on a trans-
plant waiting list.
Discussion
AS is a severe condition with multiple manifestations.
Published cases indicate an association between silico-
sis (not only accelerated) and increased levels of some
Figure  2.  (A) Alveolar septa thickened with lymphohistiocytic infil-
trates and early fibrosis; haematoxylin–eosin stain, scale bar = 200 µm.
(B) Optically active crystals in both the interstitium and the alveolar
lumen; polarized light microscopy, scale bar = 100 µm.
484  OCCUPATIONAL MEDICINE
tumour markers. This may be attributed to secondary
alveolar lipoproteinosis. In primary pulmonary lipopro-
teinosis, increases in CYFRA 21-1, carcinoembryonic
antigen, CA 19-9, CA 15-3 and tissue polypeptide anti-
gen (TPA) have been observed in both serum and BALF
[4,5]. In both our patients, CYFRA 21-1 and NSE were
elevated, as were thymidine kinase and TPA in the previ-
ously reported case and CA-125 in the present case [2].
Fang et al. [6] demonstrated significantly increased serum
levels of CA-125 and NSE in individuals with silicosis
without malignancies. This is consistent with findings in
the present case. Unlike both our studies, the authors
failed to show significantly increased CYFRA 21-1 [6].
Particularly in recent years, increasing attention has
been paid to an association between inhalational exposure
to silica and various systemic autoimmune diseases. A sig-
nificant link between silica exposure and the risk for devel-
oping granulomatosis with polyangiitis (odds ratio 3.56,
95% confidence interval 1.85–6.82) was shown by Gómez-
Puerta et al. [7] in their meta-analysis of six case-control
studies. Silica exposure has also been linked to increased
levels of autoantibody production, immune complexes and
excess production of immunoglobulin, even in the absence
of the full clinical features of a distinct autoimmune dis-
ease [8]. Similarly, increased levels of ANAs and ANCAs
together with CICs were observed in the present case, but
no other signs of autoimmune disease.
In the literature, therapeutic use of whole lung lav-
age (WLL) in silicosis patients has been reported with
varying results. In our previously reported case, the func-
tional parameters and chest radiograph deteriorated fol-
lowing WLL [2]. LT is the only life-saving therapeutic
option in end-stage silicosis, although it is typically a
rare indication for LT [9]. However, Singer et  al. [10]
claimed that subjects with silicosis undergoing LT had
higher survival estimates than those undergoing LT for
other occupational lung diseases.
The case represents a flagrant violation of occupa-
tional safety and health standards leading to irreversible
health effects. Despite its low incidence, awareness of the
condition needs to be raised to maintain and improve the
preventive measures.
Key points
•• Accelerated silicosis both resembles acute silicosis
and has features of chronic silicosis.
•• Although the incidence of accelerated silicosis is
very low, its prognosis is poor. Accelerated sili-
cosis can be largely prevented by health and safety
measures.
•• Silicosis and its accelerated form is connected with
an elevation of tumour markers. Strong evidence
also suggests an association with rheumatoid dis-
ease markers.
Funding
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This work was supported by the Internal Grant Agency of
Palacký University Olomouc (IGA LF_UP 2017_014, IGA
LF_UP 2018_015) and the University Hospital Olomouc
Fund (RVO 00098892).
Competing interests
None declared.
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