Systemic sclerosis due to crystalline silica exposure among jewelry workers in Korea

Jae Yoon Kim,Sang Yoon Do,Young Hoon Moon,Chul Gab Lee,Yun Sung Kim,Byung Soon Choi,Eun-A Kim,Han Soo Song 대한직업환경의학회 2017 대한직업환경의학회지 Vol.29 No.-

Background: Occupational exposure to crystalline silica is a potential risk factor for various systemic autoimmune diseases including systemic sclerosis. The etiology of systemic sclerosis is not conclusively known, but there are epidemiological studies that show the relationship between exposure to crystalline silica and risk of systemic sclerosis. Here we report, for the first time, two cases of crystalline silica-related systemic sclerosis in patients who worked in crystal processing in the jewelry-manufacturing field. Case presentation: Case 1 is a 57-year-old man who had worked mainly in crystal processing for multiple jewelryprocessing companies for 17 years, since the age of 15 years. He contracted tuberculosis at the age of 25 years and showed Raynaud’s phenomenon of both the hands and feet at age 32 years. Digital cyanosis and sclerosis developed at approximately age 41 years. The patient was diagnosed with systemic sclerosis at age 48 years. Case 2 is a 52-year-old man who worked in crystal processing for various jewelry-processing companies for 7 years, since the age of 23 years. He first showed signs of cyanosis in the third and fourth digits of both hands at age 32 years, was diagnosed with Raynaud’s syndrome at age 37 years, and was diagnosed with systemic sclerosis at age 38 years. Crystal processing is a detailed process that involves slabbing and trimming the selected amethyst and quartz crystals, which requires close proximity of the worker’s face with the target area. In the 1980s and 1990s, the working hours were 12 h per day, and the working environment involved 15 workers crowded into a small, 70-m2 space with poor ventilation. Conclusion: Two workers who processed crystals with a maximum crystalline silica content of 56.66% developed systemic sclerosis. Considering the epidemiological and experimental evidence, exposure to crystalline silica dust was an important risk factor for systemic sclerosis. An active intervention is necessary to reduce exposure in similar exposure groups in the field of jewelry processing.

Sjogren 증후군을 동반한 전신성 진행성 경화증 1 예

김병수(Byoung Soo Kim),이규백(Kyu Back Lee),이은주(Eun Joo Lee),김해랑(Hae Rang Kim),조원용(Won Yong Cho),김형규(Hyoung Kyu Kim),원남희(Nam Hi Won) 대한내과학회 1989 대한내과학회지 Vol.37 No.5

N/A Progressive systemic sclerosis is an uncommon connective tissue disease marked by increases in fibrotic connecfive tissue with scarring, fibrosis and vascular obliteration of the skin, GI tract, lungs, heart and kid-ney. Therefore, the clinical manifestation is widely varied in extent and severity, and the etiology is not well known although it is suspected to belong to the category of autoimmune disease. For this reason, there is difficulty in the diagnosis of progressive systemic sclerosis. Progressive systemic sclerosis is usually associated with other connective tissue diseases, but progressive systemic sclerosis with Siogren's syndrome is rarely reported in Korea. The occurrence of Siogren's syndrome in progressive systemic sclerosis would further validate its inclusion in the group of autoimmune diseases. We experienced a 50-year-old female who had xerophthalmia, xerostomia, Raynaud's phenomenon and scleroderma with cellulitis on the right lower leg and pneumonia on both lower lung fields. We diagnosed progressive systemic sclerosis based on Raynaud's phenomenon, skin manisfestations and biopsy, and diagnosed Si5grens syndrome based on xerostomia, xerophthalmia, and submaxillary gland biopsy. With supportive care for cellulitis and pneumonia, the patients condition improved and then we controlled the progressive systemic sclerosis and Sidgrens syndrome with symptomatic treatment for xerostomia, xerophthalmia and Raynaud's phenomenon, & prednisone with cytoxan. Therefore, we reported a case of progressive systemic sclerosis with Sjogren's syndrome and briefly reviewed the related literature.

결체조직질환 환자에서 조갑 융기부 (Nailfold) 모세혈관 소견의 임상적 유용성에 관하여

송광선 ( Kwang Seon Song ),이수곤 ( Soo Kon Lee ),홍천수 ( Chein Soo Hong ) 대한류마티스학회 1994 대한류마티스학회지 Vol.1 No.1

레이노 현상은 혈관의 가역적 경축에 의한 것으로 설명되고 있으며, 전신성 경화증 환자의 70% 이상에서 초기증세로 호소하고 있다. 이러한 환자에서 모세혈관의 변화는 질환의 정도와 내부 장기의 침범여부를 예측하는데 도움이 될 것으로 보고 되었고 그 모세혈관의 변형을 관찰하기 위해서는 조갑융기부가 적당하다. 저자등은 결체조직 질환중 전신성 경화증의 진단 및 진행범위 추정에 도움이 되는 지를 관찰하기 위하여, Widefield stereomicroscope을 이용하여 전신성 경화증 환자 11예, 레이노병 환자 6예 그리고 전신성 홍반성 낭창 환자 10예 등 총 27예의 조갑 융기부를 관찰하였다. 레이노 현상의 기간은 평균 9.6년(1-40년)이었으며, 발병 당시의 연령은 평균 34.2세(14-63세)이었다. 모세혈관 변화 정도는, 전신성 홍반성 낭창 환자에서 class 1이 7예(70%), class 2가 3예이었고 레이노병 환자는 class 1이 7예(70%), class 2가 3예이었고 레이노병 환자는 class 1이 3예(50%), class 2가 2명(33%) class 3가 이었다. 전신성 경화증 환자에서는 class 1이 없고 class 2가 2예(18%), class 3이상이 9예(82%)으로 전신성 경화증 환자에서 병변의 정도가 더 심하였다. 수지 병변의 정도와 모세혈관의 변형정도와는 전신성경화증에서 유의한 상관관계를 보였으나(r=0.71, p<0.01), 다른 두 질병에서는 이들에 비하여 낮은 상관성을 보여 주었다. 전신성 경화증 환자에서 장기 침범수와 조갑융기부 모세혈관 변화와의 관계를 보면, 장기 침범의 증거가 없었던 1예가 class 3의 모세혈관 병변을 보여 준 것을 제외하면, 한개의 장기를 침범한 3예에서 class 2와 4, 두개의 장기를 침범한 5예서 class 3과 5, 세개의 장기를 침범한 2예에서 class 4를 보이고 있어 장기 침범이 많을수록 모세혈관의 변형이 심한 것으로 나타났다(p=0.03). 이상의 결과로 조갑융기부 모세혈과 소견이 전신성 경피증 환자에서 진단적 가치가 있고, 병변의 범위를 예측하는데 도움이 될 것으로 생각한다. Objective: Raynaud`s phenomenon is a vascular disorder characterized by reversible spasm of arteries of fingers. It is the first symptom in 70% of partients with systemic sclerosis. The more systemic involvment the worse prognosis is expected in patients with systemic sclerosis. A more reliable indication of systemic sclerosis is the microvascular involvement by the disease (characteristic patterns of capillary abnormality in the nailfold). Our puroses were to evaluate the significance of the degree of nailfold capillary abnormlitry in making the diagnosis fo systemic sclerosis, and in determining organ involvement in patients with systemic sclerosis. Methods: Twenty-six patients with Raynaud`s phenomenon whose diagnosis were systemic sclerosis (10 patients), SLE (10 patient), Raynaud`s disease (6 patients) were observed for nailfold capillary abnormalities by widefield microscopy. Results: Capillary abnormalities were seen in 100% of the systemic sclerosis (10 patients), 30% of the SLE (3 patients) and 50% of the Raynaud`s disease (3 patients). A significant correlation between degree of finger lesions (r=0.718) or organ involvement (X2=20.4, p=0.015) and capillary abnormality class was found although a significant correlation was not found between the duration of the disease and the degree of capillary abnormality in patients with systemic sclerosis (r=0.32). Conclusions: Nailfold capillary abnormality can easily be observed and could be used as an assistive tools for the diagnosis and prediction of prognosis and extent of organ involvement in patients with Raynaud`s phenomenon especially in patients with systemic sclerosis.

전신성 경화증의 임상상에 관한 연구

강성욱(Seong Wook Kang),이윤종(Yun Jong Lee),차훈석(Hoon Seok Cha),김현아(Hyun Ah Kim),박명희(Myung Hee Park),오명돈(Myoung Don Oh),송영욱(Yeung Wook Song),최강원(Kang Won Choi),이은봉(Eun Bong Lee),한창완(Chang Wan Han),백한주(Han J 대한내과학회 1999 대한내과학회지 Vol.57 No.6

N/A Background : Systemic sclerosis (scleroderma) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and internal organs. It is remarkably heterogeneous in initial presentations and internal organ involvement. Limited and diffuse cutaneous subsets of systemic sclerosis (SSc) are known to be different in clinical and laboratory features. The aim of the present study was to determine the clinical characteristics of systemic sclerosis in Koreans. Methods : Fifty-six patients with systemic sclerosis at the Rheumatology Clinic of Seoul National University Hospital were studied for age, sex, symptoms, signs, and laboratory results. The differences in clinical and laboratory features between limited and diffuse cutaneous subsets were investigated. Results : The mean age at diagnosis of 56 patients (male:female=1:4.6) was 42.4 years (range 11-72 years). The patients consisted of 30 limited and 26 diffuse cutaneous SSc. Cutaneous involvement was as follows: sclerodactyly (100%), Raynaud's phenomenon (94.6%), digital pitting scar (66.1%), subcutaneous calcinosis (1.8%). In musculoskeletal system, 25 cases (46.3%) developed arthralgia/arthritis, 14 cases (25.9%) myalgia. In gastrointestinal system, esophagus was affected in 11 cases (20.7%). Respiratory involvement consisted of interstitial lung disease (24 cases, 43.7%) and pulmonary hypertension (2 cases, 3.6%). Total skin score and functional vital capacity showed significant negative correlation (p<0.05). Cardiovascular involvement consisted of congestive heart failure (3 cases, 5.5%) and pericardial effusion (1 case, 1.8%). Azotemia was found in one patient (1.8%). Antinuclear antibody was positive in 53 cases (94.6%) and anticentromere antibody 2 cases (3.6%). Anti-Scl 70 antibody was positive in 46.4% of all patients, 40.0% of limited scleroderma and 53.8% of diffuse scleroderma. When comparing clinical features between limited and diffuse cutaneous subsets, musculoskeletal involvement was more common in limited scleroderma. Conclusion : Systemic sclerosis in Koreans showed various systemic and organ involvement, musculoskeletal system, lung and esophagus being commonly affected. There was no significant difference between limited and diffuse scleroderma in clinical features except musculoskeletal involvement. Investigation of major internal organs, especially lung and esophagus, is needed, regardless of cutaneous subsets in systemic sclerosis. (Korean J Med 57:979-987, 1999)

A case of ischemic ulcers in systemic sclerosis

( Byeol Han ),( Sook Ja Son ),( June Hyunkyung Lee ),( Tae Young Han ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Systemic sclerosis (SSc) is a chronic autoimmune disease of the connective tissue characterized by vasculopathy and organ fibrosis. In addition to life-threatening organ involvement, skin ulcers are important complications severely affecting the quality of life and activity of daily living in SSc. Especially digital ulcers are considered as a sentinel sign for early organ involvement in very early diagnosis of SSc. A 59-year-old woman presented with 3-month history of persistent multiple digital ulcers on her fingers tip. She had episodes of Raynaud phenomenon, with severe pain, numbness, and cyanosis when exposed to the cold. Also, multiple ulcers with crust and livedo reticularis along the both lower legs were noted. Laboratory evaluation revealed a positive antinuclear antigen of 1:320 and a strongly positive anti-scl-70 antibody confirming the diagnosis of diffuse systemic sclerosis. Histopathologic examination from ischemic ulcerative lesions on the finger tip and lower leg showed ulcer and prominent increased collagen bundles which is consistent with systemic sclerosis. Treatment with systemic vasodilators, cyclophosphamide, glucocorticoid and closed dressing led to resolution of skin lesions. Herein, we report a case of ulcerative lesions in systemic sclerosis. Ischemic ulcerative skin lesions especially on the digits should be evaluated whether it is related to autoimmune disorders such as systemic sclerosis.

전신성 경피증에 동반된 갑상선 기능저하증과 부갑상선 기능저하증 1예

이상엽,한진,안원석,박미경,이성원,김덕규,정원태 대한내과학회 2002 대한내과학회지 Vol.63 No.6

Systemic sclerosis is associated to lymphocytic infiltration and fibrosis of multiple organs. The cause of systemic sclerosis is autoimmune disorder, so systemic sclerosis may be associated with other connective tissue disease or endocrine disease which may be induced by autoantibody. In this case, we experienced 44 year-old woman who presented with sclerosis on hand and upper arms. In serum, tri-iodothyronine (T3), Ca^+2 and parathyroid hormone (PTH) are in high concentration, thyroid stimulating hormone (TSH) and phosphate are in low concentration. She is diagnosed to hypothyroidism and hypoparathyroidism in systemic sclerosis. Hypothyroidism and hypoparathyroidism in systemic sclerosis may be rare. We report the case of hypothyroidism and hypoparathyroidism in systemic sclerosis with review of literature.

신증후군을 동반한 전신성 홍반성 루푸스-전신성 경화증 중복 증후군

윤보영 ( Bo Young Yoon ),이찬희 ( Chan Hee Lee ),한성훈 ( Seong Hoon Han ),이윤우 ( Yun Woo Lee ) 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.2

Renal manifestations of systemic sclerosis are proteinuria, hypertension, azotemia and renal crisis. Mild proteinuria is common, but the nephrotic syndrome occurring in association with systemic sclerosis has been rarely reported. The majority of nephrotic syndrome cases are caused by glomerulonephritis in patients with an overlap syndrome of SLE and systemic sclerosis. This report suggests that the nephrotic syndrome can be an infrequent sequel of systemic sclerosis alone without glomerulopathy. A 38-year old woman was suffering from visual loss and headaches. She had Raynaud`s phenomenon and thickened skin on nearly whole body. Urine protein was 4,950 mg/day and serum albumin level was 2.7 g/dL. The result of renal biopsy was renovasculopathy-compatible with systemic sclerosis.

한국인 전신성 경화증의 임상상과 자가항체에 관한 연구

박석규 ( Suk Kyu Park ),김태환 ( Tae Hwan Kim ),전재범 ( Jae Bum Jun ),정성수 ( Sung Soo Jung ),배상철 ( Sang Cheol Bae ),김신규 ( Think You Kim ),김성윤 ( Seong Yoon Kim ),유대현 ( Dae Hyun Yoo ) 대한류마티스학회 2001 대한류마티스학회지 Vol.8 No.4

Objective: The aim of present study was to determine the differences in clinical features, laboratory data and autoantibody profile between limited and diffuse subsets of progressive systemic sclerosis in Korea, and to see differences from foreign studies on the same disease. Methods: Fifty-six patients with systemic sclerosis at the Hospital for Rheumatic Diseases were studied for age at diagnosis, subtype, clinical features, laboratory data, systemic involvement and pattern of autoantibodies by careful review of medical records and interviews. Results: The disease was relatively more prevalent in female groups of third, forth and fifth decade (male:female=1:17.7, mean age: 39.5, range: 11-68 years). The patients consisted of 29 diffuse and 27 limited subsets. Clinical features were as follows: sclerodactyly (92.9%), Raynaud`s phenomenon (92.9%), dyspnea (71.4%), arthralgia/arthritis (64.3%), dry cough (58.9%), myalgia (51.8%), rale (48.2%), esophageal symptoms (41.1%), digital pitting scar (41.1%), telangiectasia (28.6%). Antinuclear antibody was positive in all 54 cases (100%) which is tested. The respiratory system involvement was statistically higher in diffuse subset than in limited subset (77.4 vs. 52.0%, p=0.046). The anti-centromere antibody was detected in 1 case with limited subset and the anti-Scl-70 antibody in 6 cases (11.3%) with diffuse subset. Conclusion: Interstitial lung disease was more prevalent in patients of this study than patients of other countries. Investigation of pulmonary system is needed, especially in earlier stage and diffuse type. Systemic sclerosis-related autoantibodies (anti-Scl 70 antibody, anti-centromere antibody) are less prevalent than the results of other studies.

면역글로불린 치료 후 호전된 전신경화증 위장관 침범 1예

최경진 ( Kyoung Jin Choi ),황정화 ( Jeong Hwa Hwang ),김현숙 ( Hyun-sook Kim ),이준성 ( Joon Seong Lee ) 대한내과학회 2017 대한내과학회지 Vol.92 No.2

전신경화증 환자는 위장관 침범을 하는 경우가 많으나 효과적인 치료법은 부족하다. 저자 등은 식도 내압 검사, 24시간 산도 검사 및 설문조사를 통해 광범위 전신경화증 환자의 위장관 침범을 확인하였고 면역글로불린을 투여하여 치료하였다. 이는 국내에서는 드문 증례이므로 문헌고찰과 함께 이를 보고하는 바이다. Systemic sclerosis is an intractable clinical subset characterized by diffuse fibrosis, vasculopathy, and immune abnormalities. Diffuse cutaneous systemic sclerosis has a variety of symptoms depending on the internal organs involved, such as the lungs, heart, kidneys, and gastrointestinal system. Gastrointestinal involvement is frequent and one of the major cause of morbidity and mortality. There is no sustained, effective therapy for treating the active gastrointestinal involvement of systemic sclerosis. Although immunoglobulin is commonly used in the treatment of other autoimmune diseases, its effects in the treatment of the gas-trointestinal involvement in systemic sclerosis are unclear. Here, we report a patient with a severe cough caused by uncontrolled gastrointestinal involvement in diffuse cutaneous systemic sclerosis treated with immunoglobulin. (Korean J Med 2017;92:213-218)

대장암과 동반된 폐 침범이 있는 전신경화증 1예

이기성,김재승,송영주,황원태,김대정,이창수,길세용,조현정,최정우,강성환,김호정 중앙대학교 의과대학 의과학연구소 2012 中央醫大誌 Vol.37 No.3

Systemic sclerosis is a chronic systemic disorder of unknown etiology characterized by thickening of the skin and distinctive involvement of multiple internal organs. The association of systemic sclerosis and cancer is not clear. However many studies have noted an increased risk of cancer among patients with systemic sclerosis. We report a 62-year old male who had systemic sclerosis and Bormann type II adenocarcinoma of sigmoid colon. The patient also was diagnosed as interstitial lung disease on the computed tomography.