Erasmus syndrome – silicosis and systemic sclerosis

Miranda, Aryádine Allinne Machado de; Nascimento, Alice Carvalho; Peixoto, Isy Lima; Scrignoli, Juliana Alves; Cardoso, Maria do Socorro de Lucena; Ribeiro, Sandra Lúcia Euzébio

doi:10.1016/s2255-5021(13)70038-5

Cited by 10 publications

(3 citation statements)

References 9 publications

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“…Egg shell calcification (ESC) is also rarely reported in SSc, but prominent hilar and/or mediastinal ESC, especially in a male patient, should always raise suspicion of ES, probably the only clue to the diagnosis of ES as other changes in CXR or HRCT are indistinguishable from idiopathic SSc. [ 17 ] All our patients had ILD with a predominance of NSIP pattern. One patient in the ES group had PIH in addition to ILD; however, it was not known whether it was primary or secondary to ILD.…”

Section: Discussionmentioning

confidence: 97%

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

Sharma,

Gupta

2024

Indian Journal of Dermatology

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Background: Systemic sclerosis is an autoimmune connective tissue disease characterized by fibrosis in skin and internal organs. Chronic exposure to silica may not only lead to silicosis of lungs but also systemic sclerosis. Systemic sclerosis is relatively commoner in females; however, occupational exposure to silica in males makes them vulnerable to silica--associated systemic sclerosis (Erasmus syndrome). Objective: To describe the clinico-epidemiological aspects of systemic sclerosis in males in a retrospective cohort study. Materials and Methods: The data were analysed retrospectively for demographic profile and clinical characteristics including examination findings, laboratory investigations, and treatments of all male patients diagnosed with systemic sclerosis with or without silica exposure, managed from January 2018 to December 2021. Results: Eight out of twelve patients were having silica exposure in the form of stone cutting, cement exposure, and working with concrete. The average age was 55 ± 10.72 years with average smoking exposure of around 24.4 ± 12.8 pack years. Skin thickening was reported by all patients with an average modified Rodnan score of 18.33/51 in diffuse and 7/51 in limited cutaneous systemic sclerosis. Raynaud’s phenomenon and sclerodactyly were universal findings, while 9 (75%) patients had digital pitted scars. Antinuclear antibodies were present in all patients and specific antibodies substantiated the clinical assessment in almost all patients. Interstitial lung disease was the most common systemic finding present in 11 (91%) patients and tuberculosis was diagnosed in 2 (25%) cases with silica exposure. Gastrointestinal and cardiac system involvement was seen in 5 (41.6%) and 4 (33.3%) patients, respectively. Conclusion: Systemic sclerosis in males against the gender predilection indicates the role of occupational exposure. Silicosis and systemic sclerosis synergistically add to lung damage, and at the same time, these patients are more prone to infections like tuberculosis.

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Section: Discussionmentioning

confidence: 97%

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

Sharma,

Gupta

2024

Indian Journal of Dermatology

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“…In end-stage disease, lung transplantation is a treatment option for selected patients. 3,10 We add this case to the small number of published case reports of silicosis and SSc co-existing in the same patient, [11][12][13][14][15] with the aim of raising awareness to this association, and as a reminder that the same environmental and occupational exposures may be a risk factor for different diseases.…”

Section: Discussionmentioning

confidence: 99%

Erasmus Syndrome: An Underrecognized Entity

et al. 2022

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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.

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“…Ten of them had necrosis of the fingertips, 16 pulmonary fibrosis and 9 esophageal involvement (14). Only few other cases of Erasmus syndrome are described in the literature (15)(16)(17)(18)(19)(20)(21)(22). Besides affecting workers in the gold (1) and coal (2, 14) mines, Erasmus syndrome was also described in other professionals, such as sculptors and individuals exposed to abrasive powders (4), drillers (17), quartz crushing worker (22) and dental technicians (20,21).…”

Section: Case Reportmentioning

confidence: 99%

Erasmus syndrome in a marble worker

et al. 2016

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Erasmus syndrome is defined as the association of silica exposure and subsequent development of systemic sclerosis. The limited number of cases reported in the literature mainly involves miners and only sporadically other professionals. We describe a case of Erasmus syndrome in a marble worker. A 68 year old man came to our observation complaining pelvic and scapular girdle pain, evening fever, intense weakness and emaciation for about 1 month. He also reported to have had Raynaud's phenomenon in his hands for the last 13 years. Also, his occupational history revealed a chronic exposure to silica dust. The patient presented pain in his shoulders and hips, moderate skin thickening and sclerosis in his hands and fingers extending proximally to his wrists. The diagnosis of systemic sclerosis was determined according to his clinical and medical history, the positivity of anti-Scl 70 antibodies, the nailfold capillaroscopy suggestive of an active scleroderma pattern and the detection of a mild restrictive pulmonary syndrome. The evaluation of the organbased complications excluded a gastroenterological and cardiovascular involvement, while the chest computed tomography (CT) detected multiple small nodules with a mantle distribution and enlarged lymph nodes with no signs of interstitial lung disease and fibrosis. Additional tests (positron emission tomography-CT, flexible bronchoscopy and broncho-alveolar lavage) excluded infectious diseases and cancer. However, given the pulmonary involvement, we performed a histological examination of the parenchyma and lymph nodes, which revealed a picture of pneumoconiosis. In the end, the occupational history and the findings from the diagnostic procedures led to the diagnosis of pulmonary silicosis. The precise definition of the pulmonary involvement was essential to the therapeutic approach to this patient.

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Erasmus syndrome – silicosis and systemic sclerosis

Cited by 10 publications

References 9 publications

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

Erasmus Syndrome: An Underrecognized Entity

Erasmus syndrome in a marble worker

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