Focal vasculitic myositis as a primary manifestation of Behçet’s disease: a case series of 10 Korean patients in a locomotive medicine clinic

Abstract Objectives Muscle involvement in Behçet’s disease (BD) is rare, and several cases have been reported in the literature. Therefore, this study aimed to describe the clinical, laboratory and imaging findings in adult patients presenting with BD-associated myositis before the diagnosis of BD. Methods We retrospectively screened patients who visited a locomotive medicine clinic presenting with myalgia, local swelling, or tenderness of extremities without an established diagnosis of BD. We enrolled patients whose pain in the extremities was proven to be suggestive of focal vasculitic myositis and who were eventually diagnosed as having BD at the initial visit or during follow-up. We thoroughly reviewed the clinical, histological and imaging findings and treatment outcomes in patients who presented with focal vasculitic myositis as the primary manifestation of BD. Results Ten adult patients with focal vasculitic myositis as the primary manifestation of BD were enrolled. The lower and upper extremities were affected in eight and two patients, respectively. The affected lower extremities were the calf (n = 6) and thigh muscles (n = 2). The common findings of MRI included high signal intensity of the affected muscles and intermuscular fascia on fat-suppressed images, suggestive of myofascitis and oedematous changes in the subcutaneous layer. The results of skin or muscle biopsy were suggestive of vasculitis. All the patients were pain-free at the short-term follow-up (1–3 weeks) after oral steroid therapy. Conclusion Focal vasculitic myositis can be a primary manifestation of BD warranting medical attention. BD-associated myositis responds well to oral steroid therapy.

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Neuro-Behcet's Disease: Initial and Follow-up MR Imaging Findings

Journal of the Korean Radiological Society ◽

10.3348/jkrs.1998.39.3.469 ◽

1998 ◽

Vol 39 (3) ◽

pp. 469

Author(s):

Chan Sung Kim ◽

Sun Seob Choi ◽

Ha Jong Lee ◽

Dong Ho Ha ◽

Yong Il Lee

Keyword(s):

Mr Imaging ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Imaging Findings ◽

Behcet's Disease

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Faculty Opinions recommendation of Long-term efficacy of infliximab in refractory posterior uveitis of Behcet's disease: a 24-month follow-up study.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.9582962.10243060 ◽

2011 ◽

Author(s):

Carlos Pavesio ◽

Claire Hooper

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Posterior Uveitis ◽

Behcet's Disease ◽

Follow Up Study ◽

Term Efficacy

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AB0531 GOLIMUMAB IN THE TREATMENT OF SEVERE AND/OR REFRACTORY VASCULO-BEHÇET’S DISEASE: A SINGLE-CENTRE EXPERIENCE IN CHINA

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.2272 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1562.1-1563

Author(s):

L. Sun ◽

J. Liu ◽

W. Zheng

Keyword(s):

Aortic Regurgitation ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

High Dose ◽

Efficacy And Safety ◽

Behcet's Disease ◽

Severe Aortic Regurgitation ◽

Tnf Α

Background:Vascular involvement is one of the leading causes of mortality and morbidity in Behcet’s Disease (BD)1. Surgical treatments are difficult for Vaculo-BD (VBD) patients due to the high risk of serious postoperative complications without effective and promptly perioperative immunotherapy2, 3. Anti-tumor necrosis factor alpha (TNF-α) therapy has been reported as a potential treatment in severe VBD, e.g. infliximab (IFX) and adalimumab (ADA). However, only few case reports are available regarding the fully humanized monoclonal antibody to TNF-α, golimumab (GOL), in the management of VBD4.Objectives:The objective of this study was to report the efficacy and safety of GOL for the treatment of severe and/or refractory VBD.Methods:We retrospectively analyzed the efficacy and safety profile of patients with severe and/or refractory VBD treated with GOL in our medical center between 2018 to 2020.Results:Nine VBD patients (8 male and 1 female) were enrolled, with a mean age and median course of 37±8.6 years and 72 months (range 12 to 300), respectively. Cardiac involvements (severe aortic regurgitation secondary to BD) were presented in 7 patients, including 2 patients with post-operative paravalvular leakage (PVL) after aortic valve replacement surgery. Multiple vascular lesions were documented in the other 2 patients, including one patient with life-threatening multiple pulmonary aneurysms, pulmonary thromboembolism and recurrent deep vein thrombosis, and another patient with abdominal aortic pseudoaneurysm and multiple artery stenosis and occlusion. Prior to GOL therapy, all patients experienced disease progression despite high-dose glucocorticoids combined with multiple immunosuppressants. Moreover, seven patients required effective and fast control of inflammation and a decrease of glucocorticoid dose during the perioperative period. They were treated with GOL, 50mg every 4 weeks, in combination with background low-or medium-dose glucocorticoids and immunosuppressants, for a median of 6 (range 3-15) months. After a mean duration of follow-up of 10 (range 2-6) months, all patients achieved improvement both in clinical symptoms and serum inflammation markers. The ESR level [4.88±4.94 mm/h vs 31.13±31.78mm/h, P<0.01] and CRP level [1.9 (0.11-3.73)mg/L vs 24.3 (0.4-85.57)mg/L, P<0.01] significantly decreased. The dosage of glucocorticoid[10 (0-15) vs 40 (0-100)mg/d, P<0.01] effectively tapered, indicating a potential steroid-sparing effect. No newly-onset aneurysm and recurrent venous thrombosis were observed. Also, one patient had a marked reduction in size and number of pulmonary aneurysms. No post-operative PVL was observed in the five patients after Bentall operation with a median follow-up of 10 months. One patient with severe aortic regurgitation remained stable and without surgical intervention with the treatment of GOL for 16 months. No severe complication occurred in one patient after underwent endovascular repair of abdominal aorta for 8 months. GOL was well-tolerated, and no serious adverse event was observed.Conclusion:Our results suggested that GOL is safe and effective for the treatment of patients with severe and / or refractory VBD. Further controlled studies are warranted to confirm the therapeutic potential of GOL in VBD patients.Disclosure of Interests:None declared

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THU0326 TOFACITINIB IN THE TREATMENT OF SEVERE AND REFRACTORY BEHÇET’S DISEASE: A SINGLE-CENTRE EXPERIENCE IN CHINA

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.2802 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 393.1-393

Author(s):

J. Liu ◽

L. Sun ◽

F. Zhang ◽

W. Zheng

Keyword(s):

Rheumatoid Arthritis ◽

Autoimmune Diseases ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Efficacy And Safety ◽

Jak Inhibitors ◽

Gastrointestinal Involvement ◽

Behcet's Disease ◽

Intestinal Ulcers

Background:Small-molecule JAK inhibitors have succeeded in the treatment of rheumatoid arthritis, psoriasis, and inflammatory bowel disease1. Tofacitinib is under investigation for various autoimmune diseases2, but its effectiveness on Behçet’s disease(BD) has not been demonstrated.Objectives:We aimed to investigate the efficacy and safety of Tofacitinib in the treatment of severe and refractory BD.Methods:We retrospectively analyzed the efficacy and safety profile of Tofacitinib in treating severe and refractory BD patients in our hospital from 2017 to 2020.Results:Thirteen BD patients (7 males and 6 females) were enrolled, with a mean age and median course of 40.6±14.7 years and 84 months (60, 132). Vascular/cardiac, gastrointestinal, and articular involvement were presented in 5, 6, and 2 patients, respectively. Three patients had multiple arterial stenosis or occlusion, two presented with aortic root dilation with aortic valve regurgitation, and one experienced perivalvular leakage (PVL). All the six patients with gastrointestinal involvement had multiple episodes of ileocecal and colon ulcers, intestinal bleed, and three had anastomotic ulcers or leaks.All the patients had received high-dose glucocorticoids and immunosuppressants before tofacitinib therapy, they displayed poor response with evidence of disease progression; furthermore, three patients with gastrointestinal involvement and one patient with polyarthritis had failed anti-TNF antibody treatment. They were then treated with Tofacitinib, 5mg twice daily, with background glucocorticoids and immunosuppressants, for a median of 6 months (range 4 to 19).After a median follow-up of 7 (5, 19) months, the ESR and CRP level decreased significantly (21(8, 50) mm/h vs 8(3, 19.5) mm/h, P<0.01, and 25(5.85, 49.5) mg/L vs 1.89(0.44, 6.65) mg/L, P<0.01, respectively). All patients with vascular/cardiac and articular involvement achieved clinical improvement. Vascular lesions of three patients were radiologically stable, no progressive aneurysm or PVL was observed. Two patients with intestinal ulcers revealed complete mucosal healing; the other three had sustained elevation of ESR and CRP, active mucosal ulcers, recurrent bleeding, or fistula formation. The dose of corticosteroids was tapered in six cases (46.2%), furthermore, the number of immunosuppressants lessened in seven cases. However, two patients had herpes zoster infection during follow up, while being treated with five to six immunosuppressants in addition to Tofacitinib for refractory intestinal ulcers.Conclusion:Our study suggests that Tofacitinib is effective for the treatment of vascular and articular BD; given the limited data, its therapeutic effect on gastrointestinal BD could not be validated. We have to be cautious of infectious risk for severely immunocompromised patients. Further large-scale prospective studies are warranted to confirm the therapeutic potential of JAK inhibitors in BD patients.References:[1]Banerjee, S., Biehl, A., Gadina, M., Hasni, S. & Schwartz, D. M. JAK–STAT Signaling as a Target for Inflammatory and Autoimmune Diseases: Current and Future Prospects. Drugs 77, 521–546 (2017).[2]Fragoulis, G. E., Mcinnes, I. B. & Siebert, S. JAK-inhibitors. New players in the field of immune-mediated diseases, beyond rheumatoid arthritis. Rheumatol. (United Kingdom) 58, i43–i54 (2019).Disclosure of Interests:None declared

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Surgical management of a giant left distal pulmonary artery aneurysm caused by Behcet’s disease

Interactive Cardiovascular and Thoracic Surgery ◽

10.1093/icvts/ivaa030 ◽

2020 ◽

Vol 30 (6) ◽

pp. 943-944

Author(s):

Xiaobing Li ◽

Xian Fan ◽

Li Shen ◽

Rufang Zhang

Keyword(s):

Pulmonary Artery ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Rare Complication ◽

Median Sternotomy ◽

Artery Aneurysm ◽

Pulmonary Artery Aneurysm ◽

Behcet's Disease

Abstract Pulmonary artery (PA) aneurysm is a very rare complication of Behcet’s disease. We report on a 14-year-old boy with a giant left distal PA aneurysm caused by Behcet’s disease. A left thoracotomy was first performed to separate the aneurysm, but it was interrupted due to continuous and massive tracheorrhagia. We immediately converted to a median sternotomy and established cardiopulmonary bypass (CPB). The patient’s condition was stable; aneurysmectomy and left-down lobectomy were successfully performed. Results of the 2-year follow-up were favourable. Based on our experience, we recommend selecting CPB when performing surgery on patients with PAA, especially those with Behcet’s disease.

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