Fifty-seven-year-old man
Digital necrosis and contracture associated with swelling in cold weather, cyanosis, and tingling (Fig. 1). 
The patient worked in crystal processing in the jewelry-processing industry for 17 years from the age of 17 years in 1976 to January 1994. In 1984, he was first diagnosed with pulmonary tuberculosis, which recurred but was cured in 2000. He first experienced Raynaud's phenomenon of both the hands and feet in 1991. He had a chest tube inserted in 1993 due to left pneumothorax. In approximately the year 2000, he developed digital cyanosis and hardening. In April 2006, he reported swelling, pain, and gangrenous lesions of the right great toe and left third finger. He subsequently presented to a local university hospital, where he was first diagnosed with systemic sclerosis. His symptoms progressed to digital contracture and auto-amputation of the distal phalanx of the left third finger due to necrosis. He suffered from a generalized tonic-clonic seizure (GTCS) in September 2006.
Non-smoker
Non-drinker
The patient worked in crystal processing for the Iksan Precious Metal Complex for approximately 17 years, from 1976 to 1994. He stopped working at approximately age 35 years when he first developed symptoms and has not worked since.
Upon presentation in August 2015, the patient was found to have amputation of the distal phalanx of the left third finger and limited movement of the first and second phalanges due to sclerodactyly. He was also found to have telangiectasia of the hands and face.
According to the results of the laboratory tests conducted on September 1, 2015 in rheumatology department of our hospital, the white blood cell (WBC) count, red blood cell (RBC) count, and platelet count were within normal limits. Thyroid function tests were within normal limits, with free thyroxine (T4), 1.76 ng/dL; triiodothyronine (T3), 81.5 ng/dL; and thyroid-stimulating hormone (TSH), 1.14 muIU/mL. The erythrocyte sedimentation rate (ESR) was elevated to 52 mm/h (normal <20 mm/h), and C-reactive protein (CRP) was also elevated, at 3.09 mg/dL (normal <0.3 mg/dL). Anti-Scl (scleroderma)-70 antibody was positive at 106.8 U/mL (reference range < 15 U/mL). Fluorescent antinuclear antibody (FANA) was positive with a fluorescent intensity of 4+, a homogenous type and a titer of 1:640. Other autoimmune markers such as rheumatoid factor, anti-CCP antibody (anti-cyclic citrullinated peptide antibody), and anti-centromere antibody were negative. According to the medical records brought by the patient, FANA (homogenous + nucleolar type) and anti-Scl-70 were positive in 2006.
In the simple chest radiograph taken at a different center in 2011, there was evidence of pulmonary tuberculosis sequelae, with observed bilateral lung volume loss, fibrosis scarring and distortion, and bronchiectasis with cicatrization. The patient was diagnosed with combined pulmonary fibrosis with emphysema(CPFE) after observing a reticular pattern in both the lower lungs and pleural thickening and calcification of the right lower lung. In the simple chest radiograph taken at our hospital in 2016, we observed emphysematous bullae in both the upper lungs, associated with cicatricial changes as well as fibrocalcification of both the upper lungs and the right lower lung. The reticular pattern and number of bullae increased since 2011, showing an overall deterioration (Fig. 2). 
Echocardiography performed in our hospital showed mild pulmonary hypertension with a pulmonary artery systolic pressure (PASP) of 51 mmHg. Nailfold capillary microscopy showed capillary atrophy, which suggests chronic-stage systemic sclerosis. Hand perfusion scintigraphy showed mildly decreased perfusion and blood pooling of the chilled finger to about 81% compared to the opposite finger.
The patient showed classic sclerodactyly of the hands, digital tip ulcers and pitting scars, and telangiectasia. He also showed abnormal nailfold capillaries as well as mild pulmonary hypertension by echocardiography. He was diagnosed with interstitial lung disease as per radiological evidence, and he had a past medical history consistent with Raynaud's syndrome. He also showed decreased perfusion by hand perfusion scintigraphy. He was anti-Scl-70 antibody positive. Based on the above findings, he fulfilled one major criterion (proximal scleroderma) and three minor criteria (sclerodactyly, digital pitting scars of the fingertips or loss of substance of the distal finger pad, bilateral basilar pulmonary fibrosis) according to the American Rheumatism Association (ARA) criteria (1980). Based on scoring using the American College of Rheumatology/European League Against Rheumatism Collaborative Initiative (ACR-EULAR) Classification Criteria for Systemic Sclerosis (2013), the patient also fulfills the diagnostic criteria, thus confirming the diagnosis of definite systemic sclerosis (Table 1). 
He is currently undergoing medical treatment in rheumatology department of our hospital every 4 weeks. The drugs he is taking are prednisolone, platelet aggregation inhibitor, angiotensin II receptor antagonist, nonsteroidal anti-inflammatory drug, xanthine oxidase inhibitor and endothelin receptor antagonist.
He was approved for industrial accident compensation from Korea Workers' Compensation & Welfare Service (KCOMWEL) for systemic sclerosis in 2016. His symptoms are no longer worse and remain similar. In winter, however, deterioration of the hardness of his fingers often occurs.