Systemic Sclerosis Associated with Occupational Exposure to Solvents

1. Abstract

Background:SystemicSclerosis(SS)isararemultisystemicdisorderwithchangesintheimmunesystem, vascular and connective tissue. Furthermore, there is an increase in the synthesis and accumulation of collagen andextracellularmatrixcomponentsintheskinandinternalorgans,withrepercussionsonthelungs,gastrointestinaltract,heartandkidneys.SSoccursinsusceptibleindividualsandistriggeredbypoorlyunderstoodfactors. Thereisevidenceofgeneticsusceptibility,howeverstudiesshowunder5%ofagreementbetweenmonozygotic twins,pointingthatenvironmentalfactorsplayaroleinthisimmunologicaldysregulation.Since1950therehave been reports of occupational risk factors in SS development, such as infectious agents, neoplasms, exposure to vinyl, silica, metals and solvents. Methods:WereportacaseofayoungmanwhodevelopedSSafterworkingformorethan15yearsexposed to various solvents. Results:ThemechanismbywhichsolventsactinthepathogenesisofSSremains unclear. Conclusions: It is likely that many occupational SS have not had the causal relationship recognized, thus, probably a review of the occupational exposures would lead to a greater identification of the environmental relationtoSS,withabetterknowledgeofthetoxicagentsinvolvedinitsonsetleadingtotheexclusionofthese substances from the professional environment.

2. Key words

Scleroderma- Solvents- Scleroderma; Systemic- Occupational Disease

3. Introduction

Systemic Sclerosis (SS) is a rare multisystemic disorder with changes in the immune system, vascular and connective tissue. There is presence of autoantibodies,CD4+perivascularinflammatoryinfiltrate,increasedex- pression of adhesion molecules in vessels and interstitium; alteration of microvasculature with endothelial damage, reduction of capillaries and arterial thickening, producing an obstructive vasculopathy. Furthermore, there is an increase in the synthesis and accumulation of collagen and extracellular matrix components in the skin and internal organs, with re- percussions on the lungs, gastrointestinal tract, heart and kidneys [1, 2]. Theprogressionofthediseaseisvariablerequiringsequentialandsimul- taneous dysfunction of various regulatory mechanisms. The incidence of SS is 4.5 to 18.7 new cases per million inhabitants in Europe and the United States [3]. Since 1950 there have been reports of occupational risk factors in SS de- velopment [1]. Epidemiological evidence of several environmental risk factors has already been analyzed [1], such as infectious agents, neo- plasms, exposure to vinyl, silica, metals and solvents [4]. Theseexposuresareoftenoflongdurationandthedegreeofexposureis

4. PatientsandMethods

DMS, 38 years old, male, white, assembler in a mechanical workshopfor 17 years. His main activities were paintings and general mechanics, with daily exposure to oil, inks, glues, gasoline, kerosene, thinner and others solvents. He received individual protective equipment, such as hand cream, rubber boots, shirt, pants, leather shoe, protector headset, cotton jacket, nitrile glove and leather glove. He used to work in a shed, windowless, with poor ventilation. About 7 years ago he started to present reddish spots on the skin of his arms,legsandtrunk,associatedwithdifficultyswallowinganddizziness. Heperformedaskinbiopsy,diagnosedwithscleroderma.Twoyearslater, hestartedwithjointpain,whatledtotheuseofmethotrexate(MTX),10 mg per week. Five years later, he started to present high blood pressure and was diagnosed with hypertension. Currently he uses MTX 15mg per week, lanzoprazol, zolpiden, cloxazolan, clobazan and maxopran. He has difficulty to swallow, heartburn and an important gastro-esophageal reflux that forces him to sleep with several pillows. He maintains joint and cervical pains despite the treatment.

5. Physical Exam

Good general condition. Eupneic. Rare brownish macules isolated in the trunk, upper and lower limbs, with slight atrophy (Figure 1). Hands with slight thickening of skin digits (Figure 2). Eurostar-01 Scale Score.

6. List ofTests

Histopathology 10/2011- Lumbar skin- compatible with scleroderma. Esophagealmanometry12/2011-Loweresophagealsphincterhypotonia. Chest Tomography 11/2013- Normal. Esophagealmanometry11/2013-Ineffective,moderateesophageal motility. Laboratory 10/2014- Anti DNA: Native negative. Lupus anticoagulant: positive. Esophageal scintigraphy 10/2016 - Normal. Echocardiography10/2016-Normal.Spirometry11/2016-Normal. Laboratory 08/2017- Anti DNA- Native 1:10; VHS 3.

7. Discussion

Autoimmune diseases include dozens of different diseases characterized by pathological auto reactive immune responses and high associated morbidity and mortality [1, 8]. SSoccursinsusceptibleindividualsandistriggeredbypoorlyunderstood factors[9].Prevalencerangesfrom50to300permilliondependingon The mechanism by which solvents act in the pathogenesis of SS remains unclear[2].Theyarewidelyusedinvariousactivitiessuchasdrycleaning (tetrachlorethylene), painting (thinners, toluene), enamel removers and glue solvents (acetone, methyl acetate, ethyl acetate), stain removers (hexane)[2],beingpresentincountlessprofessions,suchasmanufacturing andrepairingofvehiclesengines,ships,aircraft;tireproduction,plastics, varnishes,paints,enamels,cements,adhesives,oils,footwear,animaland synthetic leather goods, metals; in the paper and dye industry, printing works,laundries,amongothers[2].Ourpatientreportedcontinuous and chronic occupational exposure to various types of solvents, paints and chemical diluents for about 17 years, which is compatible with the profession of vehicle mechanic. However, even with the diagnosis ofthe disease a few years ago, he had never been told to avoid contacts or change profession. Interestingly, the fact that the disease is autoimmune seems to induce doctors to ignore their relation to work.

8. Conclusions

Exposure to solvents is clearly and strongly associated with SS developmentandshouldberecognizedasanoccupationaldiseasewhenit occurs at the workplace. Consideringthelongexposureofourpatienttoagentsinvolvedintheonset ofSS,wediscusstheneedtoinvestigateexposuretooccupationalagents inthisdiseaseinordertopreventtheoccurrenceofmoresevereformsof the disease, as well as to implement changes in the work environment.

References

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2. MARIE I, GEHANNO, J F Environmental risk factors of systemic sclerosis. Semin Immunopathol, v. 2015; 463-73.

3. ALAYA, Z. Silica-associated systemic sclerosis occurring after an occupational exposure to arc welding]. Pan Afr Med J. 2016; 25.

4. MARIE I. Systemic sclerosis and exposure to heavy metals:Acase control study of 100 patients and 300 controls. Autoimmun Rev. 2017; 16: 223-230.

5. SHARMARK,SHARMAAK,SHARMAA.ErasmusSyndrome: AssociationofSilicosisandSystemicSclerosis.IndianDermatol Online J. 2018; 185-187.

6. JAINS.ErasmusSyndrome:SilicosisandSystemicSclerosis.Indian J Occup Environ Med. 2017; 94-96.

7. CHIFFLOT,H.Incidenceandprevalenceofsystemicsclerosis:a systematic literature review. SeminArthritis Rheum. 2008; 223-35.

Citation:

Lise MLZ. Systemic Sclerosis Associated with Occupational Exposure to Solvents. Annals of Clinical and Medical Case Reports 2020.