Two Cases of Refractory Thrombocytopenia in Systemic Lupus Erythematosus that Responded to Intravenous Low-Dose Cyclophosphamide

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/µL. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/µL after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.ope

Clinical Manifestations of Korean Adult Patients with Henoch-Schonlein Purpura

OBJECTIVE: We investigated the clinical data and analyzed the significant prognostic factors for outcomes in Korean adult patients with Henoch-Schonlein Purpura (HSP). METHODS: We retrospectively reviewed the medical records of 52 patients over 20 years-old, who visited the Yonsei University Severance Hospital from December 1999 to November 2009, and fulfilled the classification criteria for HSP. We investigated the epidemiologic data, clinical features, renal biopsy findings, laboratory results and disease outcomes. RESULTS: The median age was 43.5 (20~83) years old and 29 out of 52 patients (55.8%) were male. HSP exhibited seasonal variation and most frequently developed in winter (42.3%), followed by spring (25.0%). Upper respiratory infection was the most common known preceding event for HSP development. Skin manifestations were observed in all subjects, followed by kidney (80.8%), gastro-intestine (57.7%) and joints (26.9%). After a median follow-up period 14.5 (1~227) months, 12 patients experienced HSP relapse (23.1%), and 7 patients had chronic renal failure (13.4%). Univariate analysis showed that renal insufficiency (p=0.002) and nephritic syndrome (p=0.026) at diagnosis were significantly related to the development of chronic renal failure. Of the two parameters, only initial renal insufficiency was found to be a significant predictive value for chronic renal failure (OR=28.7, p=0.001, 95% confidential interval 3.6~225.3). CONCLUSION: Renal insufficiency at diagnosis may be a useful predictive factor for progression to chronic renal failure in Korean adult patients with HSP.ope

Overlap Syndrome of Antisynthetase Syndrome and Rheumatoid Arthritis: A Case Report

The antisynthetase syndrome is characterized by anti-Jo-1 antibody production, interstitial lung disease, inflammatory muscle disease, and, in many cases, fever, polyarthritis, Raynaud’s phenomenon, and mechanic’s hands. Joint signs and symptoms occur in up to 90% of patients with antisynthetase syndrome, occasionally as the initial manifestations. Although visual inspection of the hands shows changes that are highly suggestive of rheumatoid arthritis, notable differences exist. Antisynthetase syndrome is a predominantly nonerosive arthropathy with subluxations of the distal interphalangeal joints. It manifests as overlap syndrome with other connective tissue diseases. However, overlap syndrome of antisynthetase syndrome and rheumatoid arthritis is rare. We treated a 51-year-old male patient with overlap syndrome of antisynthetase syndrome and rheumatoid arthritis, and report the case with a review of the literatureope

Differences in Clinical Manifestations and Outcomes between Adult and Child Patients with Henoch-Schonlein Purpura

We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55.4% vs 27.1%), while adults had a greater frequency of diarrhea (20% vs 1.6%). Anemia, elevated C-reactive protein, and level of IgA were more frequently observed in adults (25% vs 7.1%, 65.6% vs 38.4%, 26.3% vs 3.5%). Renal involvement in adults was more severe than in children (79.2% vs 30.4%). Chronic renal failure showed a significant difference in outcomes of HSP between adults (10.4%) and children (1.8%) after a follow up period of an average of 27 months. Furthermore, renal insufficiency at diagnosis was significantly related to the progression to chronic renal failure. Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients.ope

A Case of Atypical Giant Cell Arteritis Presenting as a Fever of Unknown Origin

Giant cell arteritis (GCA) is a systemic vasculitis predominantly found in individuals of Northern European ancestry over 50 years of age. Typically it presents with new-onset persistent headache, claudication of jaws, and existence of an abnormal temporal artery. However, the diagnosis of GCA and the assessment of its activity remain challenging, especially in patients presenting with a variety of non-specific symptoms and laboratory tests. In those cases, 18F-fluorodeoxy-glucose positron emission tomography (18F-FDG-PET) is useful for the diagnosis of GCA. Recently, the number of foreign patients who visit domestic hospitals for medical care is increasing in Korea. Here-in, we report a Russian patient who was admitted to our hospital with fever of unknown origin (FUO). FUO study was performed to search for infection or malignancy and GCA was suspected by 18F-FDG-PET. The patient was eventually diagnosed with GCA by random temporal artery biopsy.ope

Typical 18-FDG-PET/CT Findings of Polymyalgia Rheumatica: A Case Report

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by generalized pain and morning stiffness in the shoulders, hip girdle, and neck. Since the pathogenesis of PMR is still uncertain, the diagnosis of PMR depends on clinical features. There have been several studies regarding radiological tools for the diagnosis of PMR. Recent studies using 18-FDG-PET showed bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis in PMR patients. However, there was no report on the efficacy of 18-FDG-PET for diagnosis of PMR in Korea. Here, we are first reporting a case of a Korean patient with PMR, who had radiological findings including bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis on 18-FDG-PET/CT.ope

단핵세포계 세포주 THP-1에서 osteoclastogenesis inhibitor의 활성에 관한 연구

학위논문(박사)--서울대학교 대학원 :치의학과 구강미생물학전공,1998.Docto

Clinical manifestations of Korean adult patients with Henoch-Schonlein Purpura

의학과/석사[한글]Henoch-Schonlein Purpura (HSP) 는 소아에서 흔하게 나타나는 혈관염이나 성인에서도 드물게 나타날 수 있다. 한국 성인 HSP 환자군을 대상으로 특징을 분석한 연구는 거의 없는 상태로, 본 연구에서는 한국 성인 HSP 환자군을 대상으로 임상 양상을 분석하고 예후와 관련된 인자를 조사하고자 하였다. 연세대학교 의과대학 세브란스 병원에서 1999년 12월부터 2009년 11월까지 HSP로 진단 받고 입원 또는 외래 치료하였던 20세 이상의 환자 52명을 대상으로 의무기록을 통한 후향적 연구를 시행하였다. 역학적 특성, 임상양상, 신조직검사, 검사실 소견, 예후에 대하여 조사하였다. 대상 환자는 총 52명(남자 29명, 여자 23명)으로 남:녀 비는 1.26:1이었고, 평균연령은 43.5세이었다. 계절에 따른 발병율은 겨울(42.3%), 봄(25%)에 높았다. HSP의 발병 선행 요인 중에 상기도 감염이 가장 높은 비율을 차지하였다. 진단 당시 피부 병변은 100%에서 관찰되었고, 신장 침범 80.8%, 위장관 침범 57.7%에서, 관절 침범 26.9% 순으로 관찰되었다. 평균 14.5(1-227)개월의 추적관찰 후 12예(23.1%)에서 재발이 있었고, 7예(13.4%)는 만성신부전으로 진행하였다. 만성신부전으로 진행하는 데에 기여하는 인자를 단변량 분석을 통하여 추정하였을 때, 진단 당시 신부전(p=0.002) 및 신증후군(p=0.026)의 동반 여부에 따라 만성신부전으로 진행한 군과 그렇지 않은 군에 통계적으로 유의한 차이를 보였다. 다변량 분석에서, 진단 당시 동반된 신부전은 만성신부전으로의 진행을 예측하는 독립적인 인자로 확인되었다. (OR=28.7, p=0.001, 95% confidential interval 3.6~225.3) 성인 HSP 환자에서 진단 당시 신기능 부전을 보이는 환자의 경우 예후가 좋지 않을 가능성이 높음을 고려하여, 좀더 적극적인 치료와 추적관찰이 필요할 것으로 생각된다. [영문]ope

Application of the 2010 ACR/EULAR classification criteria for rheumatoid arthritis in Korean patients with undifferentiated arthritis

OBJECTIVE: The aim of this study was to determine how many patients with undifferentiated arthritis (UA) are classified as patients with rheumatoid arthritis (RA) by the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for RA. METHODS: The 2010 ACR/EULAR criteria for RA were applied to 102 patients with UA. UA is defined as an inflammatory arthritis that does not meet any criteria for a definitive diagnosis. We analysed discrepancy in the classification between previous criteria and the 2010 criteria by identifying patients who were categorized as those with RA. RESULTS: The mean age of the patients was 46.8 ± 14.3 years. Rheumatoid factor (RF) was positive in 36 patients (35.2%), and 30 patients (29.5%) were positive for anti-cyclic citrullinated peptide antibody (anti-CCP). The 2010 ACR/EULAR criteria classified 33 patients (32.4%) as having RA, and 31 of them (93.9%) had the involvement of 1-3 small joints. All patients were seropositive, and 25 of them (75.8%) had high positive RF or anti-CCP. Seropositivity and small joint involvement was significantly different between patients who were classified with RA and those who were not (p < 0.001). CONCLUSION: Using the 2010 ACR/EULAR criteria, 32.4% of patients with UA were classified as having RA, and all were seropositive. Most of the UA patients with high positive RF or anti-CCP could be classified as having RA when we applied the 2010 ACR/EULAR criteria.ope