Cement Factory Worker Presenting With Raynaud Phenomenon, Breathlessness, and Digital Ulcers

Case PresentationA 54-year-old man who had worked in a cement factory for the past 30 years, presented to chest clinic with complaints of insidious onset, gradually progressive breathlessness intermittent dry cough three years’ duration. The symptoms were associated bluish discoloration fingers on exposure cold. He also gave history digital ulcers at fingertips same These used heal, leaving behind pitted scars. There was an tightening skin involving face, extremities, and trunk. complained food getting stuck throat, he take frequent sips water while eating, along feeling early satiety. pruritus. no arthritis, rash, or alopecia. been treated 15 years ago pulmonary TB, 9 months anti-tubercular therapy. denied any similar illness family. On eliciting his occupational history, revealed that other coworkers workspace illness. nonsmoker teetotaller known addictions chemicals. A General physical examination normal pulse BP, tachypnea (respiratory rate, 26 breaths/min) hypoxemia (saturation 88% room air). pallor, clubbing (grade II), bilateral pedal edema (Fig 1A), raised jugular venous pressure. Dermatological over trunk, modified Rodnan Skin Score 21 (max score, 51), hyperpigmentation lower limbs, sclerodactyly 1A). fingertip serosanguinous discharge tuft resorption. Examination respiratory system fine inspiratory crackles. Third heart sound heard auscultation, abdomen tender hepatomegaly. Investigations anemia (Hb = 8.9 g/dL). Peripheral blood smear showed microcytic hypochromic red cells, presence anisocytosis, pencil target cells. metabolic biochemical profile normal. anti-nuclear antibody nucleolar pattern immunofluorescence. extractable nuclear antigen positive anti-topoisomerase antibody. radiograph multiple nodular opacities preponderance mid zones left all right side 1B). Spirometry restrictive defect, impaired diffusion studies (diffusing capacity lungs carbon monoxide 40%). ECG sinus tachycardia atrial enlargement ventricular hypertrophy. underwent 2D echocardiography, which suggestive severe hypertension (right systolic pressure 62 + pressure). CT usual interstitial lung fields fibrosis volume loss, predominant 1C). Arterial gas analysis pH 7.384, pCO2 34.7 mm Hg, PaO2 54.5 bicarbonate (HCO3−) 20.9 Hg. What is diagnosis? Diagnosis: Silicosis systemic sclerosis (Erasmus syndrome) earliest pneumoconiosis be have described literature attributed inhalation silica (silicon dioxide) crystalline form. Silica ubiquitous nature health hazard, especially when earth’s crust disturbed as result industry, during excavatory activities such rock mining. chronic form occurs after prolonged many usually presenting complaints, dyspnea cough, our case. Scleroderma (SSc) inflammatory-fibrotic disease autoimmune origin. It characterized by deposition excessive extracellular matrix visceral organs. SSc manifests features microvascular injury internal hallmark this thickening tightness underlying subcutaneous tissue. can involve organ systems, it inflammation resultant degeneration integument, involvement heart, lungs, kidney, GI tract, synovium. are subtypes described: diffuse form, limited sine scleroderma commonly involved subtype. collagen results endothelial damage. Also, arteries their luminal obliteration, contributes hypertension. Erasmus, 1957, first describe higher prevalence South African gold miners. patients dust occupation, shown strong risk factor development SSc, men. has significantly increase average years. silicosis-induced present insidious-onset dust. examination, they hypoxic air, auscultation reveals velcro crackles, diagnosis confirmed radiologically, these shows basal infiltrates, reduced volumes being detected spirometry. peri-bronchiolar distribution, centrilobular nodules tree-in-bud appearance, well areas ground-glass opacities. patient radiological findings. case using 2013 ACR/EULAR Classification Criteria SSc. point system-based criteria, wherein various aspects tightening, ulcers, telangiectasia, Raynaud phenomenon, disease, arterial hypertension, scleroderma-related antibodies scored individually, total score nine required classify 21. thickening, either neck, distal extremities (called limited), may proximal trunk diffuse). method measuring validated clinical trials. calculated estimating thickness upper arm, forearm, hands, fingers, thighs, legs, feet both sides body, addition anterior chest, abdomen, thus making 17 sites. Each site individually from 0 3, based severity, thereby arriving maximum 51. pinched index finger thumb two thumbs, given follows: (normal thickness), 3 (hide-bound skin), 1 indicating mild 2, more degree tautness. This scoring prognostic value, because high scores prone renal crisis. Chronic lead obstructive airway attributable mechanisms direct cytotoxicity causing reactive oxygen nitrogen radical species generation, release pro-inflammatory cytokines, chemokines, fibrogenic factors. factors cause emphysema. particles epithelial cell injury, facilitates entry into smaller airways, further damage localized fibrosis. Patients developing PAH, cutaneous subset. PAH denotes poor prognosis. multifactorial, group-I World Health Organization (WHO) variety, (pulmonary vasculopathy), group III hypoxia. third mechanism postulated, develops myocardial diastolic dysfunction (group II). Right catherization standard confirming PAH; however, did not consent it. In silicosis-related management comprises immune suppressants corticosteroids, cyclophosphamide, azathioprine. phenomenon managed non-dihydropyridine calcium channel blockers nifedipine, phosphodiesterase type 5 inhibitors tadalafil, pro-kinetic agents dysmotility. Topical nitrates applied ulcers. To date, proven curative treatment available silicosis. Steroids role contrary, TB. However, empirical bronchodilators mucolytics considered significant airflow obstruction. started diuretics failure, pantoprazole prokinetics hydroxychloroquine sulphate. hypoxia individuals, hence needs treated. multifactorial helpful perform catheterization ascertain contribution toward offered catheterization, but unwilling undergo procedure possible complications explained him. Thereafter, pros cons empiric vasodilator therapy discussed patient, wanted try view symptoms. Because WHO functional class IV dyspnea, commenced combination (tadalafil) endothelin-receptor antagonist (bosentan) good response improvement exercise tolerance management. 1.Exposure pneumoconiosis; rarely reported sclerosis.2.Silicosis only manifestations act trigger multi-system connective tissue disease.3.Besides affecting workers coal mines, Erasmus syndrome professionals sculptors, individuals exposed abrasive powders. Workers quartz crushing workers, drillers, dental technicians Financial/nonfinancial disclosures: None declared. Other contributions: CHEST authors ensure Journal policies report information met.