Erasmus Syndrome: An Underrecognized Entity

Authors

  • Ana Magalhães Pulmonology Department. Hospital de Santa Marta. Centro Hospitalar Universitário Lisboa Central. Lisboa.
  • Inês Moreira Pulmonology Department. Hospital de Santa Marta. Centro Hospitalar Universitário Lisboa Central. Lisboa.
  • Sofia Pinheiro Internal Medicine Department. Hospital Santo António dos Capuchos. Centro Hospitalar Universitário Lisboa Central. Lisboa.
  • Alexandra Borba Pulmonology Department. Hospital de Santa Marta. Centro Hospitalar Universitário Lisboa Central. Lisboa.

DOI:

https://doi.org/10.20344/amp.16896

Keywords:

Connective Tissue Diseases, Lung Diseases, Interstitial, Silicosis, Scleroderma, Systemic

Abstract

We present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud’s phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Chest-computed tomography revealed upper lobe consolidation. After extensive evaluation, the multidisciplinary interstitial lung disease team concluded that the patient also had advanced silicosis. After a year, there was significant clinical, radiologic, and functional deterioration of the lung disease. The patient was referred for lung transplant. Silica inhalation is the cause of silicosis but is also implicated in the development of systemic sclerosis (Erasmus syndrome). Although they share a common risk factor, it is rare to find both diseases co-existing. We present this case of a young patient where both diseases presented aggressively in order to raise awareness to this association.

Downloads

Download data is not yet available.

References

Rimal B, Greenberg AK, Rom N. Basic pathogenetic mechanisms in silicosis: current understanding. Curr Opin Pulm Med. 2005;11:169-73.

Leung CC, Yu IT, Chen W. Silicosis. Lancet. 2012;379:2008-18.

Fernández Álvarez R, Martínez González C, Quero Martínez A, Blanco Pérez JJ, Carazo Fernández L, Prieto Fernández A. Guidelines for the diagnosis and monitoring of silicosis. Arch Bronconeumol. 2015;51:86-93.

Pollard KM. Silica, silicosis, and autoimmunity. Front Immunol. 2016;7:1-7.

Mora GF. Systemic sclerosis: environmental factors. J Rheumatol. 2009;36:2383-96.

Mccormic ZD, Khuder SS, Aryal BK, Ames AL, Khuder SA. Occupational silica exposure as a risk factor for scleroderma : a meta-analysis. Int Arch Occup Env Heal. 2010;83:763-69.

Erasmus LD. Scleroderma in gold-miners on the Witwatersrand with particular reference to pulmonary manifestations. South African J Lab Clin Med. 1957;3:209-31.

Ingegnoli F, Ughi N, Mihai C. Best practice & research clinical rheumatology update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis. Best Pract Res Clin Rheumatol. 2018;32:223-40.

Nair A, Walsh SL, Desai SR. Imaging of pulmonary involvement in rheumatic disease. Rheum Dis Clin North Am. 2015;41:167-96.

Singer JP, Chen H, Phelan T, Kukreja J, Golden JA, Blanc PD. Survival following lung transplantation for silicosis and other occupational lung diseases. Occup Med. 2012;62:134-7.

Machado de Miranda AA, Carvalho Nascimento A, Lima Peixoto I, Alves Scrignoli J, de Lucena Cardoso M do S, Euzébio Ribeiro SL. Erasmus syndrome – silicosis and systemic sclerosis. Rev Bras Reumatol. 2013;53:310-3.

Bello S, Rinaldi A, Trabucco S, Serafino L, Bonali C, Lapadula G. Erasmus syndrome in a marble worker. Reumatismo. 2015;67:116-22.

Ganguly J, Kumar A, Samanta SK, Mitra R, Kundu S. Erasmus syndrome: a case report of silicosis-induced Scleroderma in a 26-year-old male. Oman Med J. 2013;28:26-8.

Chaouch N, Mjid M, Zarrouk M, Rouhou SC, Ammous I, Hantous S, et al. Erasmus-syndrome with pseudo-tumour masses. Rev Mal Respir. 2011;28:924-7.

Chakrabarti S, Pan K. Erasmus syndrome in a 42-year-old male: a rare case report. J Clin Diagnostic Res. 2015;9:OD01-3.

Downloads

Published

2022-05-13

How to Cite

1.
Magalhães A, Moreira I, Pinheiro S, Borba A. Erasmus Syndrome: An Underrecognized Entity. Acta Med Port [Internet]. 2022 May 13 [cited 2024 Dec. 24];36(2):122-6. Available from: https://actamedicaportuguesa.com/revista/index.php/amp/article/view/16896

Issue

Section

Case Report