Systemic sclerosis due to crystalline silica exposure among jewelry workers in Korea: two case reports

Abstract

BackgroundOccupational exposure to crystalline silica is a potential risk factor for various systemic autoimmune diseases including systemic sclerosis. The etiology of systemic sclerosis is not conclusively known, but there are epidemiological studies that show the relationship between exposure to crystalline silica and risk of systemic sclerosis. Here we report, for the first time, two cases of crystalline silica-related systemic sclerosis in patients who worked in crystal processing in the jewelry-manufacturing field.Case presentationCase 1 is a 57-year-old man who had worked mainly in crystal processing for multiple jewelry-processing companies for 17 years, since the age of 15 years. He contracted tuberculosis at the age of 25 years and showed Raynaud’s phenomenon of both the hands and feet at age 32 years. Digital cyanosis and sclerosis developed at approximately age 41 years. The patient was diagnosed with systemic sclerosis at age 48 years.Case 2 is a 52-year-old man who worked in crystal processing for various jewelry-processing companies for 7 years, since the age of 23 years. He first showed signs of cyanosis in the third and fourth digits of both hands at age 32 years, was diagnosed with Raynaud’s syndrome at age 37 years, and was diagnosed with systemic sclerosis at age 38 years.Crystal processing is a detailed process that involves slabbing and trimming the selected amethyst and quartz crystals, which requires close proximity of the worker’s face with the target area. In the 1980s and 1990s, the working hours were 12 h per day, and the working environment involved 15 workers crowded into a small, 70-m2 space with poor ventilation.ConclusionTwo workers who processed crystals with a maximum crystalline silica content of 56.66% developed systemic sclerosis. Considering the epidemiological and experimental evidence, exposure to crystalline silica dust was an important risk factor for systemic sclerosis. An active intervention is necessary to reduce exposure in similar exposure groups in the field of jewelry processing.

Systemic Sclerosis in Males—Deciphering the Enigma of Erasmus Syndrome

“…There is convincing evidence that autoimmune connective tissue diseases including systemic sclerosis (SSc), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), anti-neutrophil cytoplasmic antibody (ANCA) positive vasculitis, and overlap syndromes have been associated with silicosis. [ 6 10 ]…”

“…The incidence of idiopathic SSc in women is reported to be up to 14 times higher than that in men. [ 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 ]…”

A Pathological Study of Acute Pulmonary Toxicity Induced by Inhaled Kanto Loam Powder

“…Occupational exposure to crystalline silica is associated with silicosis, lung cancer, pulmonary tuberculosis, and chronic obstructive pulmonary disease [ 11 ]. Moreover, silicosis patients often develop autoimmune diseases [ 12 , 13 , 14 ]. It has been reported that experimental chronic intratracheal exposure to crystalline silica causes granuloma formation and/or fibrosis in rats [ 15 , 16 , 17 , 18 ].…”

Erasmus syndrome: systemic sclerosis and silicosis co‐occurrence

“…In fact this name was abandoned after it was realized Bramwell discovered this association long before Erasmus . This syndrome is described in some specific industrial workers, like stonemasons, gold miners, marble workers, jewelry workers, denim sandblasting workers, ceramic workers and dental technicians . According to a large meta‐analysis of 15 case‐control studies, the odds ratio of developing SSc for the patients who were exposed to silica is denoted as 2.81 (95% CI 1.86‐4.23; P < 0.001) .…”

“…In the treatment decision of Erasmus syndrome, severity, duration and progression rate of ILD is important, because it is the main cause of morbidity and mortality. In reported cases with Erasmus syndrome (methyl)prednisolone, methotrexate, endothelin receptor antagonists, acetyl salicylic acid, amlodipin/nifedipin are the medicines that have most commonly been used . In one study it was shown that SSc‐related ILD mostly progresses in the first 4 years of the disease .…”