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Silicosis and rapidly progressive systemic sclerosis

 Department of Clinical Immunology and Rheumatology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India

Date of Submission22-May-2022
Date of Acceptance16-Jun-2022
Date of Web Publication13-Jul-2022

Correspondence Address:
Pradeepta Sekhar Patro,
Department of Clinical Immunology and Rheumatology, Institute of Medical Sciences & SUM Hospital, Bhubaneswar - 751 030, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_101_22

How to cite this URL:
Harish B V, Sahoo RR, Sudhish G, Samanta J, Patro PS. Silicosis and rapidly progressive systemic sclerosis. Indian J Rheumatol [Epub ahead of print] [cited 2022 Dec 5]. Available from:

Dear Editor,

A 29-year-old male, an agriculturist by occupation presented with progressive skin thickening, intermittent dry cough, and significant weight loss of around 12 kg for the past 4 months. There was no history suggestive of Raynaud's phenomenon, exertional dyspnea, and gastro-esophageal reflux. Examination revealed the modified Rodnan skin score of 46 out of 51 and salt-and-pepper appearance of the skin [Figure 1]a. Investigations showed normal hemogram and renal and liver function tests. Inflammatory markers were raised. Creatinine phosphokinase and lactate dehydrogenase were elevated (<2 times the upper limit of normal). Antinuclear antibody by immunofluorescence revealed 4+ nuclear homogeneous pattern and immunoblot was strongly positive for the anti-Scl-70 antibodies. Radiograph of the chest posteroanterior view revealed multiple nodular opacities scattered over bilateral lung fields [Figure 1]b. High-resolution computed tomography of the thorax showed nodules with calcification in bilateral lung parenchyma [Figure 1]c and multiple enlarged lymph nodes with peripheral rim eggshell calcification [Figure 1]d, suggestive of silicosis. Two-dimensional echocardiography was normal. On further inquiry, the patient admitted working in the glass industry for 1 year, 9 years back. He was diagnosed with diffuse cutaneous systemic sclerosis (SSc) and silicosis, and started with low dose prednisolone and intravenous monthly pulse cyclophosphamide in view of rapidly progressive skin thickening.
Figure 1: Erasmus syndrome (silicosis and systemic sclerosis) presenting as (a) diffuse skin thickening, generalized hyperpigmentation, and salt-and-pepper appearance of the skin, (b) bilateral, discrete nodular opacities on chest radiograph posteroanterior view, (c) diffuse parenchymal lung nodules on coronal and (d) mediastinal lymph nodes with peripheral egg-shell calcification (arrows) on axial high-resolution computed tomography of the thorax

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Autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA) or Schoenfeld's syndrome is the proposed terminology to include immunologic phenomena following adjuvant exposure and encompasses siliconosis, the Gulf War syndrome, the macrophagic myofasciitis syndrome and postvaccination phenomena.[1] The spectrum of clinical manifestations can be heterogeneous and the concept of ASIA classically denotes conditions such as sarcoidosis, Sjogren's syndrome, undifferentiated connective tissue disease, silicone implant incompatibility syndrome, and immune-related adverse events due to checkpoint inhibitors.[2]

The adjuvant effect of silica in triggering autoimmunity is known. Exposure to crystalline silica is associated with systemic autoimmune rheumatic diseases including systemic lupus erythematosus, rheumatoid arthritis, and SSc.[3] Autoimmunity may be triggered through activation of the innate and adaptive immune system, and breakdown of self-tolerance, resulting in autoantibody production and organ damage.[3] The systematic review and meta-analysis by Rubio-Rivas et al. highlighted occupational and environmental SSc and concluded that both crystalline silica and solvents are implicated in the pathogenesis of SSc.[4] Erasmus syndrome is characterized by manifestations akin to SSc following exposure to silica, with or without associated silicosis. Pulmonary involvement and the presence of anti-Scl-70 antibodies are more frequent in silica-associated SSc, compared to idiopathic SSc.[5] The disease usually manifests after 15 years of initial exposure, although significant silica exposure even for a shorter duration can present early.[6] Our patient presented with rapidly progressive skin tightening over 4 months, although he had silica exposure 9 years back for 1 year. Overlap between silicosis, SSc, and dermatomyositis is also reported.[7] A detailed history including occupational and environmental exposure to silica should be sought in all patients with SSc for appropriate treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Shoenfeld Y, Agmon-Levin N. 'ASIA' – Autoimmune/inflammatory syndrome induced by adjuvants. J Autoimmun 2011;36:4-8.  Back to cited text no. 1
Borba V, Malkova A, Basantsova N, Halpert G, Andreoli L, Tincani A, et al. Classical examples of the concept of the ASIA syndrome. Biomolecules 2020;10:1436.  Back to cited text no. 2
Pollard KM. Silica, silicosis, and autoimmunity. Front Immunol 2016;7:97.  Back to cited text no. 3
Rubio-Rivas M, Moreno R, Corbella X. Occupational and environmental scleroderma. Systematic review and meta-analysis. Clin Rheumatol 2017;36:569-82.  Back to cited text no. 4
Rustin MH, Bull HA, Ziegler V, Mehlhorn J, Haustein UF, Maddison PJ, et al. Silica-associated systemic sclerosis is clinically, serologically and immunologically indistinguishable from idiopathic systemic sclerosis. Br J Dermatol 1990;123:725-34.  Back to cited text no. 5
Abdessemed A, Slimani S, Khaldoun N, Bougrina F, Ait Belabas N, Djoudi M, et al. AB0674 the Erasmus syndrome: Exposure to silica and systemic sclerosis. Ann Rheumatic Dis 2015;74:1123-4.  Back to cited text no. 6
Jain S, Samanta J, Vyas S, Mahajan S, Suri V, Kumar U. Triple overlap between silicosis, dermatomyositis, and systemic sclerosis: Opening up a pandora's box. J Clin Rheumatol 2021;27:e204-5.  Back to cited text no. 7


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