242 32nd World Congress of Internal Medicine (October 24-28, 2014) WCIM 2014
PS 0747 Rheumatology
A Case of Sweet’s Syndrome in s Patient with Systemic Sclerosis
Ji-Min LEE1, Tae-Han LEE1, Hye-Jin JEONG1, Chang-Nam SON1, Ji-Min KIM1, Sang- Hyon KIM1
Keimyung University Dongsan Medical Center, Korea1
Introduction: Sweet’s syndrome is an acute neutrophilic dermatosis characterized by multiple erythematous plaques or pustules, together with fever. It is often associated with autoimmune diseases such as rheumatoid arthritis (RA) and systemic lupus ery- thematosus (SLE), malignant neoplasm, or certain drugs. We present a case of a pa- tient with systemic sclerosis (SSc) who had severe multiple erythematous skin rashes.
Case: A 63-year-old woman with SSc treated with D-penicillamine and cyclophospha- mide for 4 years visited the clinic because of a whole body rash and a fever that had lasted for 4 days. Many erythematous macules and patches with central edematous elevation were present on her trunk and both extremities. Her CBC showed WBC 4,120/
μL, Hb 13.3 g/dL, platelet count 148k, and no neutrophilia. ESR and CRP were elevated to 60 mm/hr and 1.69 mg/dL, respectively. Histological examination of the skin lesion revealed scattered neutrophilic infi ltration with nuclear dust in the dermis. The abrupt onset of the erythematous skin rash and pyrexia above 38°C without any evidence of bacterial infection suggested the possibility of Sweet’s syndrome. The patient received steroid therapy (pulse and oral). The fever and skin rashes began to subside after treatment, and infl ammatory markers (ESR, CRP) decreased in normal range.
Conclusion: In Korea, several cases of Sweet’s syndrome were associated with RA and SLE. However, no cases have previously reported an association of this disease with SSc. We treated a patient who was diagnosed with prior SSc, had high fever and skin rashes, and whose diagnosis was confi rmed by skin biopsy. She was administered ster- oids and her symptoms subsided. This was a rare case, so we have presented this case report.
PS 0748 Rheumatology
Distinct Clinical Features of Elderly-Onset Systemic Sclerosis
Hyun Mi KWON1, Eun Young LEE1, Yeong Wook SONG1, Hyun-Ju LEE1, Eun Bong LEE1 Seoul National University Hospital, Korea1
Background: Systemic sclerosis (SSc) is a connective tissue disease characterized by fi brosis of skin and internal organs. Recent increase of elderly population predicts increase of elderly-onset SSc. We investigated clinical features and outcome of elder- ly-onset SSc compared with younger-onset SSc.
Methods: A total of 150 Korean patients with SSc with interstitial lung disease (ILD) were enrolled for this study. All the patients were cared at Rheumatology Clinic of Seoul National University of Hospital between 1978 and 2013 and fulfi lled 1980 ACR classifi cation criteria for SSc. Clinical features of patients with elderly-onset SSc (age at diagnosis =60 years) were compared with those of younger-onset SSc (age at di- agnosis <60 years). The information on demographic features, status of SSc-specifi c autoantibodies and involvement of internal organs, presence of pulmonary arterial hypertension (PAH), development of cancer and survival of the patients was obtained from electronic medical chart review. PAH was defi ned as systolic pulmonary arterial pressure >40 mmHg estimated by echocardiography. Extent of ILD on computed to- mography was divided into 4 grades. Chi-square test, Student t-test and log-rank test were applied as appropriate.
Results: There were 31 elderly-onset SSc and 119 younger-onset SSc patients and their mean age (standard deviation) at onset was 65.2 (5.2) years and 44.2 (10.5) years, respectively. Anti-centromere antibody and PAH was more commonly observed in elderly-onset SSc than younger-onset SSc (p=0.003 and p=0.017 respectively), while presence of anti-Scl70 antibody was less common in elderly-onset SSc (p=0.012).
Distribution of sex and skin subset, involvement of heart and kidney, and development of cancer were similar between both groups. Survival was signifi cantly shorter in el- derly-onset group (p=0.0027, by log-rank test) (Table 1).
Conclusion: Elderly-onset SSc is characterized by prevalence of anti-centromere antibodies and PAH than younger-onset SSc. Further study in other ethnic groups is warranted.
PS 0749 Rheumatology
Usefulness of Infrared Thermography on Hands and Feet in Raynaud’s Phenomenon as the Screening Tool
Mie Jin LIM1, Seong Ryul KWON1, Kyong-Hee JUNG1, Kowoon JOO1, Won PARK1 Inha University Hospital, Korea1
Background: Raynaud’s phenomenon (RP) is characterized by a three-phase color change after cold exposure. We supposed that infrared thermography could be used as screening tool as cold-induced vasoconstriction of distal extremities in RP lowers skin temperature which could be easily detected by thermography.
Methods: Fifty-seven patients with RP (primary RP, n=33; secondary RP, n=24) and 146 healthy volunteers were recruited. After acclimation to temperature of 24 ℃, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. A fi nger with lowest temperature among 2nd, 3rd and 4th fi nger was chosen as ‘the coolest fi nger’. The temperature difference of the coolest fi nger/fi rst toe was defi ned as temperature of palm/dorsum was subtracted by temperature of the coolest fi n- ger/1st toe, respectively.
Results: Temperature differences of both coolest fi ngers and fi rst toes signifi cantly differed between patients with RP and controls. They successfully distinguished male patients with RP from male controls but temperature difference of the coolest fi nger from non-dominant hand did not differ between female patients with RP and female controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2 ℃) differentiated patients with RP from controls (sensitivity/specifi city : 65–68 %/ 57–62 %, respectively). Temperature differences of fi rst toe (cutoff value: 3.11 ℃) discriminated patients with RP (sensitivity/specifi city : 72–74 %/ 64–68 %). Thermographic assessment of the coolest fi nger and fi rst toe combined was highly effective (sensitivity/ specifi city : 88–90 % / 44–45 %), espe- cially in men (sensitivity/ specifi city : 88 %/ 58–62 %). However, in women, thermo- graphic assessment of fi rst toe was solely suffi cient (sensitivity/specifi city : 88–90 % / 31-36 %).
Conclusions: Thermographic assessment of the coolest fi nger and fi rst toe is useful as the screening tool for RP.
PS 0750 Rheumatology
Application of the 2013 ACR/EULAR Classifi cation Cri- teria for Systemic Sclerosis to Patients with Raynaud’s Phenomenon
Jinsu PARK1, Minchan PARK1, Jungsik SONG1, Yongbeom PARK1, Sookon LEE1, Sangwon LEE1
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Ko- rea1
Background: We investigated how many patients with Raynaud’s phenomenon (RP), but not systemic sclerosis (SSc) would be reclassifi ed as SSc, and we analyzed the predictive values for the reclassifi cation as SSc using the 2013 ACR/EULAR classifi ca- tion criteria
Methods: We consecutively enrolled 64 patients with RP and 60 patients with SSc.
We applied the new classifi cation criteria to patients with RP, reclassifi ed them, and compared variables between those who were newly reclassifi ed as SSc and those who were not or previously diagnosed as SSc. The predictive values for the reclassifi cation as SSc were also assessed.
Results: Seventeen of 64 patients (26.5%) with RP were newly classifi ed as SSc ac- cording to the 2013 ACR/EULAR classifi cation criteria. Patients newly classifi ed as SSc had sclerodactyly, digital tip ulcer, telangiectasia, abnormal nailfold capillaries and the presence of anti-centromere antibody more frequently than those not. The independ- ent predictive values for the reclassifi cation as SSc were sclerodactyly (OR 60.025), telangiectasia (OR 13.353) and the presence of anti-centromere antibody (OR 11.168).
Telangiectasia and anti-centromere antibody in newly classifi ed patients were more frequently than those in previously diagnosed SSc patients.
Conclusions: 26.5% of patients with RP were newly classifi ed as SSc by the 2013 ACR/EULAR classifi cation criteria, and sclerodactyly, telangiectasia, and the presence of anti-centromere antibody were the independent predictive values for the reclassifi - cation as SSc.
