Erasmus syndrome: Association of silicosis and systemic sclerosis

Sharma, Reena; Sharma, Ashish; Sharma, Abha

doi:10.4103/idoj.idoj_201_17

Cited by 12 publications

(4 citation statements)

References 6 publications

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“…[ 10 ] Silica may have a fundamental pathogenic role in the otherwise scarce cases of male SSc as the majority of patients with SSc exposed to silica are males (96% according to systemic review and meta-analysis) due to its occupational relevancy. [ 6 14 ]…”

Section: Discussionmentioning

confidence: 99%

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: 99%

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

Sharma,

Gupta

2024

Indian Journal of Dermatology

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“…There are controversies regarding the use of PPL for IH in patients with PHACE(S) syndrome, due to fear of ischemic vascular accident (ischemic stroke). However, as it is a rare syndrome, there are few cases of ischemic stroke in the literature after using this medication and these events were related to severe arteriosus and heart disease [3,8,9]. An echocardiogram is recommended before starting treatment, in addition to a multidisciplinary discussion (cardiology, neurology, dermatology and other health professionals).…”

Section: Systemsmentioning

confidence: 99%

The Efficacy and Safety of Oral Propranolol in Hemangioma of PHACES Syndrome: A Case Report

Kondo

2023

JDR

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PHACES syndrome is a rare condition characterized by the association of infantile hemangioma with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, heart defects, ocular changes and sternal cleft. The use of propranolol in the treatment of infantile hemangioma is well established, but in PHACES syndrome there is still controversy. We use propranolol in this syndrome with good results and safety.

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“…9 Silicosis-induced or silica-associated systemic sclerosis cannot be distinguished from idiopathic systemic sclerosis either clinically or immunologically although they have higher and severe lung involvement along with anti-Scl-70 antibody. 10 The risk is higher in miners as compared to non-miners with silica exposure. 11 Lomante, J.M.J., et al 2022 reported a case of a Filipino man who developed Erasmus Syndrome after working in a grind mill for 10 years with a brief literature review of 20 patients reported in the last 10 years.…”

Section: Case Presentationmentioning

confidence: 99%

Erasmus Syndrome: A Rare Case Report of Systemic Sclerosis with Silicosis and Progressive Massive Fibrosis

Singh,

Kumar Singh

2023

Int J Sci Healthcare Res

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Erasmus Syndrome is a rare disorder that is characterized by the development of systemic sclerosis after significantly prolonged exposure to silica with or without silicosis. Systemic Sclerosis is a multisystem autoimmune disease with vascular changes, inflammation, and diffuse tissue fibrosis. We report a case of Erasmus syndrome in a 45-year-old male who worked in the marble industry. The patient presented with difficulty in breathing, skin tightening, joint pain, Raynaud’s phenomenon, microstomia, and progressive massive fibrosis. Keywords: Erasmus Syndrome, Systemic Sclerosis, Silicosis, Progressive Massive Fibrosis

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Erasmus syndrome: Association of silicosis and systemic sclerosis

Cited by 12 publications

References 6 publications

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

The Efficacy and Safety of Oral Propranolol in Hemangioma of PHACES Syndrome: A Case Report

Erasmus Syndrome: A Rare Case Report of Systemic Sclerosis with Silicosis and Progressive Massive Fibrosis

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