Scientific letter
Silicosis, sarcoidosis and systemic sclerosis in the same patient
Silicosis, sarcoidosis y esclerosis sistémica en un mismo paciente
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Specifically, interrelated cases between the following diseases have been described: sarcoidosis, systemic sclerosis and silicosis. Sarcoidosis is a systemic disease that is characterised by the presence of non-caseifying granulomas in the affected organs. Silicosis is a diffuse, chronic and incurable interstitial lung disease related to exposure to silica. Systemic sclerosis (SS) is an autoimmune disease associated with fibrosis that affects the connective tissue and endothelium. We present a clinical case of concomitant silicosis, sarcoidosis and systemic sclerosis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 39-year-old male patient, former smoker (29<span class="elsevierStyleHsp" style=""></span>pckts/year), with occupational exposure to silica, solvents and abrasives, whose medical history of interest includes insulinised type 1 diabetes mellitus without complications of target organs. He consulted because of constitutional syndrome associated with arthromyalgia, dry cough, acrocyanosis with digital ulcers and erectile dysfunction of one year of evolution. The physical examination highlighted the presence of macroscopic nail haemorrhages, digital ulcer on the second finger of the right hand, and sclerodactyly. Dry crackles were observed in pulmonary auscultation, predominantly in the left base. Of note from the blood analysis was the elevation of CK 732<span class="elsevierStyleHsp" style=""></span>U/l and aldolase 25<span class="elsevierStyleHsp" style=""></span>U/l, of the angiotensin converting enzyme (ACE) 132<span class="elsevierStyleHsp" style=""></span>U/l, as well as the presence of ANA 1/640 and anti-Scl 70 positive. Periungual capillaroscopy showed capillary dilatations and haemorrhages. Spirometry presented only a decrease in DLCO (64%), presenting desaturation in the gait test. Simple chest radiography showed a reticulonodular pattern, so the study was completed with high-resolution CT (HR-CT) in which multiple mediastinal and hilar adenopathies were observed, with nodular opacities in the upper pulmonary fields, oesophageal dilation and hepatomegaly. Similarly, the pulmonary study was completed with a bronchoscopy – in which bronchoalveolar lavage had a predominance of histiocytes and multinucleated giant cells without malignant cellularity – and a transbronchial biopsy in which non-necrotising granulomatous inflammation of the sarcoid type was observed. Given the muscular involvement, an EMG was performed in which myopathic signs were observed without neuropathy, and a muscle biopsy was carried out that showed a granulomatous myositis of the sarcoid type. The hand X-ray showed no calcinosis or distal phalange resorption. Finally, a PET/CT was performed in which multiple mediastinal and bilateral hilar adenopathies associated with centrolobular nodules in pulmonary fields were seen. Based on the clinical and histological findings, a diagnosis of systemic sclerosis (with sclerodactyly, digital ulcers, altered capillaroscopy, oesophageal dilation and anti-Scl 70 positive) was given; based on the biopsy, sarcoidosis with lung and muscle involvement, and pulmonary silicosis due to occupational exposure and the compatible imaging test.</p><p id="par0015" class="elsevierStylePara elsevierViewall">To our knowledge, the coexistence between pulmonary silicosis, sarcoidosis and systemic sclerosis has not been described in the literature. There are a large number of reported cases of SS and sarcoidosis; a diagnosis of SS coming prior to, or – to a lesser extent – concomitant with one of sarcoidosis. The majority of patients show symptoms of diffuse SS, pulmonary involvement with the presence of bilateral hilar adenopathies, reticular interstitial pattern and a decrease in DLCO, elevation of the ACE, ANA and, occasionally, anti-Scl 70 positive and presence of non-caseifying granulomas.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1–3</span></a> These findings are similar to those in our case.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The association between silicosis and sarcoidosis is less known. These are patients who have been exposed to silica that have a presence of non-caseifying granulomas with silica particles in their interior.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> These findings were identified in our patient, although they did present silicosis, given their occupational exposure to silica.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The association of SS with silica exposure is known as Erasmus syndrome. It is more frequent in men with interstitial lung disease and, often, presence of rheumatoid factor, ANA, ANCA and anti-Scl 70.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> As previously described, these characteristics are consistent with those of our patient.</p><p id="par0030" class="elsevierStylePara elsevierViewall">We can conclude that pulmonary and muscular involvement in patients with SS is not always associated with SS itself, but other diseases that may be associated with it, such as silicosis and sarcoidosis, should be considered.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Reguart Oto N, Soler Sendra A, Ortiz Santamaria V. Silicosis, sarcoidosis y esclerosis sistémica en un mismo paciente. Med Clin (Barc). 2020;154:146.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Sarcoidosis in systemic sclerosis: report of 7 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "D. Cox" 1 => "E. Conant" 2 => "L. Earle" 3 => "J. Newman" 4 => "B. Kahaleh" 5 => "S. 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