P027 : Red blood cell distribution width as a useful marker in evaluating cutaneous vasculitis

( Dae Suk Kim ),( Dong Yun Shin ),( Min Seok Lee ),( Sung Hee Kim ),( Tae Gyun Kim ),( Do Young Kim ),( Soo Min Kim ),( Min Geol Lee ) 대한피부과학회 2014 대한피부과학회 학술발표대회집 Vol.66 No.2

Background: Cutaneous vasculitis can be limited to skin or a manifestation of primary systemic vasculitis. There are no definite markers to predict systemic involvements. Higher red blood cell distribution width (RDW) is associated with disease activity in various disorders. Objectives: To evaluate whether RDW can be used as an indicator for predicting systemic disease in patients with initial cutaneous involvements. Methods: Retrospective review and analysis of clinical and laboratory parameters of 143 patients with cutaneous vasculitis. Results: The mean age was 32.0 years and 102 (64.6%) of the patients were female. Total of 132 patients were patients with primary cutaneous vasculitis and 11 were primary systemic vasculitis. Higher ratio of patients with high RDW was detected in systemic vasculitis group compared to cutaneous vasculitis group (36.4% vs 7.6%, P<0.05). The mean RDW was significantly higher in systemic vasculitis patients (P<0.05). RDW had the strongest association with systemic vasculitis (P<0.05, OR 1.834). Conclusion: In conclusion, elevated level of RDW was significantly associated with systemic vasculitis. RDW can be used as one of the marker to predict systemic disease in patients with cutaneous vasculitis.

전신성 괴사성 혈관염에서 다발성 단신경염의 임상적 고찰

김진석 ( Jin Seok Kim ),차훈석 ( Hoon Suk Cha ),서기현 ( Gi Hyun Seo ),안홍준 ( Hong Joon Ahn ),이창근 ( Chang Keun Lee ),고재현 ( Jae Hyun Koh ),김현숙 ( Hyeon Sook Kim ),최동철 ( Dong Chull Choi ),고은미 ( Eun Mi Koh ) 대한류마티스학회 1999 대한류마티스학회지 Vol.6 No.1

Background: The vasculitides are heterogeneous clinical syndromes characterized by inflammation of blood vessels. There are no pathognomonic symptoms. Mononeuritis multiplex is an acute or subacute asymmetric sensory motor syndrome involving discrete peripheral nerves and is known to occur in many illnesses including certain types of systemic vasculitis. Our purpose was to evaluate the clinical manifestations and significance of mononeuritis multiplex in systemic necrotizing vasculitis. Patients & Methods: Eight patients who were diagnosed as systemic necrotizing vasculitis at Samsung Medical Center between 1995 and 1997 were reviewed. Vasculitis was diagnosed by ACR criteria for each vasculitis and microscopic polyangiitis was diagnosed by the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitis. Three were diagnosed as Churg-Strauss syndrome, 3 as polyarteritis nodosa, and 2 as microscopic polyangiitis. In most cases, vasculitis was confirmed by a biopsy specimen showing infiltration of granulocytes, lymphocytes, and/or plasma cell into vessel walls. Occasionally, polyarteritis nodosa was confirmed by mesenteric or renal arteriography which showed microaneurysms. EMG/NCV was performed in seven patients who had neuropathy. Results: Seven of the eight patients presented with mononeuritis multiplex and one had no neuropathy. Four patients presented with neuropathy as the first symptom, and three developed neuropathy within 3 weeks after the onset of vasculitis symptoms. The peroneal nerve and sural nerve were involved in all seven patients. The median nerve, ulnar nerve and posterior tibial nerve were involved in four, two and six patients, respectively. All seven patients had lower extremity involvement. Two showed only lower extremity involvement and five had both lower and upper extremity involvement. Isolated upper extremity involvement was not seen. All patients with mononeuritis multiplex were improved by treatment with cyclophosphamide and steroid therapy. Conclusion: Mononeurtis multiplex is a common manifestation of vasculitis and occurs early in the course of systemic vasculitis. Prognosis of mononeuritis multiplex is excellent, although it may take up to one year to recover.

Whitish blotch - the most helpful dermoscopic finding of true vasculitis to select the optimal biopsy site

( Ji Yoon Choo ),( Ji Hyun Lee ),( Jun Young Lee ),( Young Min Park ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.2

Background: Cutaneous vasculitis is an inflammatory process of vessels whose diagnosis is based on the histologic evidence of endothelial damage. However, random selection of the biopsy site from lesions can’t always promise to confirm vasculitis. Objectives: To determine the dermoscopic features of ‘true vasculitis’ which may facilitate to select the optimal biopsy site. Methods: From the histopathologic data base of recent 2 years, 27 biopsy-proven cases with dermoscopic imagesavailable were finally included in this study. These cases were divided into two groups; ‘vasculitis’ or ‘vasculopathy’ group according to the histopathologic criteria for ‘true vasculitis’. Nine specimens fulfilled the standard criteria of vasculitis (‘vasculitis’ group) and 18 specimens who failed to satisfy the criteria were included in ‘vasculopathy’ group. We compared the dermoscopic images between two groups. Results: Whitish blotch was shown in 6 cases (75.0%) of ‘vasculitis’ group, whereas that in 2 (15.8%) of ‘vasculopathy’ group (P=0.006). Mottled purpuric pattern, orange-brown background, and purpuric globules were demonstrated in 100%, 66.7%, 55.6%, respectively, of ‘vasculitis’ cases, and in 83.3%, 77.8%, 50.0%, respectively, of ‘vasculopathy’ cases (P=0.495, 0.653, 1.0, respectively). Conclusion: Our results suggest the whitish blotch is the most helpful dermoscopic finding of true vasculitis to select the optimal biopsy site.

폐혈관염의 영상의학적 접근

김초희,김윤경,한정호 대한영상의학회 2021 대한영상의학회지 Vol.82 No.4

Vasculitis is a systemic disease, characterized by inflammation of the vascular wall. Although rare, it is sometimes life-threatening due to diffuse pulmonary hemorrhage or acute glomerulonephritis. Besides primary vasculitis, whose cause is unknown, numerous conditions such as autoimmune diseases, drugs, infections, and tumors can cause secondary vasculitis. Vasculitis displays various non-specific symptoms, signs, and laboratory findings; hence, diagnosis of the disease requires integration of various results including clinical features, imaging findings, autoantibody tests, and pathological findings. In this review, we have discussed the clinical, radiologic, and pathological features of vasculitis. Further, we elaborated the imaging findings and differential diagnosis of typical vasculitis that frequently involves the lung and introduced a new international classification of vasculitis, the Diagnostic and Classification Criteria in Vasculitis. 혈관염은 혈관벽의 염증을 특징으로 하는 드문 전신 질환으로 간혹 미만성 폐출혈이나 급성사구체신염으로 생명을 위협하기도 한다. 원인을 알 수 없는 일차성 혈관염도 있지만 자가면역 질환이나 약물, 감염, 종양 등 수많은 원인들에 의해 발생하는 이차성 혈관염도 있으며 분류가 복잡하고 명확한 진단검사가 없어 진단에 어려움이 있다. 또한 다양하고 비특이적인 증상과 징후 및 검사 소견을 보여 혈관염의 진단에는 임상양상, 영상 검사, 자가항체 검사, 병리소견 등 여러 가지 결과를 종합하는 것이 필수적이다. 이 종설에서는 혈관염의 분류와 진단에 중요한 증상 및 징후, 특징적으로 폐를 침범하는 대표적인 폐혈관염의 영상 소견과 감별진단, 그리고 새로운 혈관염 분류를 위한 국제적 연구인 Diagnostic and Classification Criteria in Vasculitis에 대해 소개하고자 한다.

Endoscopic Findings of Upper Gastrointestinal Involvement in Primary Vasculitis

Eun Jeong Gong,Do Hoon Kim,Joo Hyun Chun,Ji Yong Ahn,Kwi-Sook Choi,Kee Wook Jung,Jeong Hoon Lee,Kee Don Choi,Ho June Song,Gin Hyug Lee,Hwoon-Yong Jung,Jin Ho Kim,송인혜,Yong-Gil Kim 거트앤리버 소화기연관학회협의회 2016 Gut and Liver Vol.10 No.4

Background/Aims: Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. Methods: Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. Results: Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch- Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis. Conclusions: Diagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low.

A case of cutaneous vasculitis associated with granulocyte colony-stimulating factor: an unusual presentation

( Se Jin Oh ),( Young Hwan Choi ),( Hyun Jeong Byun ),( Seung Hwan Oh ),( Ji-hye Park ),( Jong Hee Lee ),( Dong-youn Lee ),( Joo-heung Lee ),( Jun-mo Yang ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Cutaneous vasculitis is a histopathologic entity that is characterized by neutrophilic transmural inflammation of the vessel wall and is associated with fibrinoid necrosis, which is also termed leukocytoclastic vasculitis. Recombinant granulocytic colony-stimulating factor (G-CSF) is clinically used for the treatment of neutropenia associated with chemoteherapy. However, vasculitis has been reported in a few cases of solid as well as hematologic malignancies and with granulocyte colony-stimulating factor therapy. A 66-year-old woman was diagnosed with multiple myeloma. The patient had been given a bortezomib treatment for 3 months and no skin lesion was developed before. Filgrastim was given to improve neutropenia. There was a rise in absolute neutrophil count and on day 14 of G-CSF, the patient developed tender and erythematous plaques on the upper chest and back. Skin biopsy revealed leukocytoclastic vasculitis. G-CSF was discontinued and complete resolution of vasculitis was achieved. Drug-induced vasculitis was usually reported to present with palpable purpura on lower extremities, unlike that in our patient who tender plaque on trunk. The aim of this case report is to highlight the importance of monitoring patients on granulocytic colony-stimulating factor therapy.

한국인 류마티스 혈관염과 안지오텐신 전환효소 유전자 삽입/결손 다형성

이경해 ( Kyung Hae Lee ),우진현 ( Jin Hyun Woo ),김숙경 ( Sook Kyoung Kim ),박용욱 ( Yong Wook Park ),이혜순 ( Hye Soon Lee ),엄완식 ( Wan Sik Uhm ),김태환 ( Tae Hwan Kim ),전재범 ( Jae Bum Jun ),배상철 ( Sang Cheol Bae ),유대현 ( 대한류마티스학회 2004 대한류마티스학회지 Vol.11 No.4

Objective: Rheumatoid arthritis has various extra-articular manifestations including rheumatoid vasculitis. Angiotensin converting enzyme (ACE) gene shows insertion/deletion polymorphism and has II, ID, DD genotypes. ACE gene is related with vasoconstriction and endothelial dysfunction in cardiovascular disease. This study was undertaken to determine the association between ACE gene polymorphism and rheumatoid vasculitis. Methods: Twenty-nine patients were collected as rheumatoid vasculitis group. DNA was isolated from blood samples collected from 114 Korean rheumatoid arthritis patients meeting American College of rheumatology 1987 revised criteria, and 114 healthy control group. Genotyping for the angiotensin converting enzyme gene insertion/deletion polymorphism was performed by polymerase chain reaction method. Results: As vasculitis manifestation, 15 patients showed neuropathy, 13 showed scleritis, 3 showed skin rash. In rheumatoid vasculitis group, II, ID and DD polymorphism was seen in 8 (27.6%), 15 (51.7%), 6 (20.7%) patients respectively and 39 (34.2%), 57 (50.0%), and 18 (15.8%) in normal controls. There was no skewing of ACE I/D polymorphism in compared with normal group. In rheumatoid arthritis control group, II, ID and DD polymorphism was seen in 37 (32.5%), 64 (56.1%), and 13 (11.4%) patients. Among rheumatoid arthritis patient, there was no significant difference between patient with vasculitis and without vasculitis. Conclusion: Our results showed that genetic polymorphisms of angiotensin converting enzyme insertion/deletion gene has no association with the susceptibility to rheumatoid vasculitis.

Treatment of refractory IgA vasculitis with dapsone: a systematic review

Lee, Keum Hwa,Hong, Sung Hwi,Jun, Jinhae,Jo, Youngheun,Jo, Woogyeong,Choi, Dayeon,Joo, Jeongho,Jung, Guhyun,Ahn, Sunghee,Kronbichler, Andreas,Eisenhut, Michael,Shin, Jae Il The Korean Pediatric Society 2020 Clinical and Experimental Pediatrics (CEP) Vol.63 No.5

IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.

CASE REPORT : Nodular Vasculitis That Developed during Etanercept (Enbrel) Treatment in a Patient with Psoriasis

( Seung-bae Park ),( In-kyu Chang ),( Myung Im ),( Young Lee ),( Chang-deok Kim ),( Young-joon Seo ),( Jeung-hoon Lee ) 대한피부과학회 2015 Annals of Dermatology Vol.27 No.5

Nodular vasculitis was introduced by Montgomery for cases of erythema induratum-like lesions that were not associated with tuberculosis. Nodular vasculitis has been associated with both nontuberculous infections and noninfectious conditions. However, there has been no report on the development of nodular vasculitis during tumor necrosis factor-α inhibitor treatment. A 28-year-old man visited our clinic for the treatment of severe psoriasis with a 20-year history. Subcutaneous injection of etanercept (25 mg, twice weekly) was started. One year later, erythematous nodules developed on his lower leg. A skin biopsy showed lobular panniculitis with extensive necrosis and vasculitis. To exclude latent tuberculosis, an assay specific for Mycobacterium tuberculosis antigens was performed, with a negative result. After stopping etanercept under the diagnosis of nodular vasculitis associated with etanercept, the lesions gradually disappeared, leaving depressed scars in 3 months. There has been no recurrence after 6 months of follow-up. (Ann Dermatol 27(5) 605∼607, 2015)

Colorectal surgical management of colitis induced by vasculitis in the absence of inflammatory bowel disease: a case report and literature review

Jessica A. Paynter,Kirby R.Qin,Georgia Seamer,Ruchira Fernando,Janelle Brennan,Chun Hin Angus Lee 대한대장항문학회 2023 Annals of Coloproctolgy Vol.39 No.3

Colitis caused by vasculitis is a rare and poorly understood pathology. Little evidence exists on its clinical presentation, path to diagnosis, and surgical management. In this report, we present a case report and literature review. A healthy 20-year-old male patient presented with hemorrhagic colitis requiring total colectomy with end ileostomy. Pathological examination showed pancolitis with multiple ulcers, transmural inflammation, hemorrhage, and microvascular thrombosis. Extensive serological testing revealed elevated cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) and eosinophilia, leading to a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) and vasculitis-induced colitis. A literature review was subsequently conducted. Nineteen studies were found documenting vasculitis-induced colitis in the absence of inflammatory bowel disease (IBD). Systemic signs of vasculitis, hemorrhagic colitis, and progression to fulminant colitis were present. Of all patients, 40.0% required colorectal surgery and 62.5% of those patients received a stoma; 25% underwent emergency surgery following failed immunosuppression. All cases relied on clinical correlation with serology and/or histopathology to reach a final diagnosis. We report a case of vasculitis-induced colitis caused by c-ANCA−positive EGPA. The review shows that vasculitis-induced colitis without IBD is an important differential that clinicians should be aware of in patients presenting with colitis.