Erasmus syndrome in a marble worker

Abstract

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.

Erasmus syndrome: Association of silicosis and systemic sclerosis

“…Erasmus syndrome is characterized by the association of exposure to silica with or without silicosis and the subsequent development of systemic sclerosis (SSc). This syndrome is named after the doctor who first described 17 cases of SSc in gold miners [ 1 ] in South Africa in 1957. A subsequent meta-analysis of 16 studies suggested that exposure to silica is a significant risk factor in the development of SSc, particularly in males.…”

Ювенільна Системна Склеродермія Й Екологія (Огляд Літератури Та Власні Дослідження)

“…Среди факторов риска развития ССД особое значение отводится экзогенному влиянию на детей высоких концентраций вдыхаемого кварца (диоксида кремния) [34]. Воздействие на организм ребенка кварца является предиктором формирования противоядерных, противогистоновых и противотопоизомеразных антител [2,6], а также антинейтрофильных цитоплазматических (направленных к миелопероксидазе и протеиназе 3) [5,24]. В патогенетических построениях частицы экзогенного кварца через активацию матриксных металлопротеиназ 1 и 3 усиливают синтез транскрипционных факторов для провоспалительных цитокинов и некоторых факторов роста [20,38].…”

Erasmus syndrome: systemic sclerosis and silicosis co‐occurrence

“…Silicosis is one of the pnuemoconioses caused by inhalation of the silica dust and results in chronic inflammation and progressive lung fibrosis especially in the upper lobes in the long term . Unfortunately, to date there is no effective treatment strategy other than symptomatic treatment …”

“…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 .…”