Efficacy and safety of anti-tumor necrosis factor α monoclonal antibodies in 16 patients with severe/refractory vasculo Behcet′s disease / 中华内科杂志

Objective:To explore the efficacy and safety of anti-tumor necrosis factor alpha (TNFα) monoclonal antibodies (mAbs) for severe/refractory vasculo-Behcet′s disease (BD).Method:The clinical data of severe/refractory vasculo-BD patients treated with anti-TNFα mAbs were retrospectively analyzed. Response of anti TNFα mAbs was analyzed. The dosage changes of glucocorticoid, the level of erythrocyte sedimentation rate (ESR) and hypersensitive C-reactive protein (hsCRP) before and after treatment were recorded, as well as side effects.Result:Sixteen patients were enrolled. Arterial lesions were reported in 12 patients, including 9 with arterial aneurysm, 6 with arterial dilation, 2 with stenosis and 2 with occlusion. Seven patients presented venous thrombosis, including lower extremity veins ( n=6), cerebral venous sinus ( n=2) and inferior vena cava system ( n=2). Two cases had both arterial and venous involvement. Before the application of TNFα mAbs, all 16 patients failed to response to prednisone or its equivalent dose of 40 (7.5-90) mg/d in combination with cyclophosphamide, methotrexate, thalidomide or azathioprine for median 4 (0-156) months. After a mean duration of treatment for (17.1±6.5) months, 15 patients achieved complete remission and 1 patient achieved partial remission. Three patients received surgery without any postoperative complications. After using anti TNFα mAbs, the dosage of prednisone [5(0-12.5)mg/d vs. 40(7.5-90)mg/d, P<0.01], ESR [(7.3±4.6) mm/1h vs. (33.5±26.7) mm/1h, P<0.01] and hsCRP [1.9(0.2-11.4) mg/L vs. 24.3(0.4-113.9) mg/L, P<0.01] were significantly decreased. Side effects were observed in 2 patients. One developed pulmonary infection 12 months after adalimumab with conventional treatment. Another patient had allergy to infliximab then switched to adalimumab. Conclusion:In combination with corticosteroids and immunosuppressants, anti-TNF α mAbs are effective and well-tolerated in severe/refractory vasculo-BD, with a favorable steroid -sparing effect and rare postoperative complications.

Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial.

Objectives: This randomized, controlled clinical trial aims to compare the efficacy and safety of glucocorticoid combined with MMF and glucocorticoid monotherapy for patients with IgG4-related disease. Methods: Sixty-nine patients newly diagnosed with IgG4-related disease were randomly divided into two groups (35 patients in Group I and 34 patients in Group II). Patients in Group I received glucocorticoid monotherapy (0.6-0.8 mg/(kg·day) and tapered gradually); patients in Group II received glucocorticoid combined with MMF therapy (1-1.5 g/day). All the patients were followed up at 1, 3, 6 and 12 months. The primary endpoint was response rate in 12 months and the secondary endpoints were relapse, remission rate and adverse reactions. Results: Group I and Group II shared almost the same efficacy at the 1 month treatment, but during the follow-up, the complete response rate in Group II was much higher than that in Group I at different time points, and the cumulative relapse rate during 1 year of therapy was much higher in Group I than that in Group II (40.00 vs 20.59%). The remission rate was lower in Group I (51.42 vs 76.47%). Relapses were more likely to happen in lung, lacrimal gland, salivary gland, paranasal sinus and kidney. MMF could reduce relapse, especially organs recurrence. No serious adverse reactions occurred in the two groups. Conclusion: Combination treatment with glucocorticoid and MMF was more effective than the monotherapy, and the relapse of IgG4-related disease might be associated with the elevated levels of serum IgG4 and the low glucocorticoid maintenance dose. Trial registration: ClinicalTrials.gov, www.clinicaltrials.gov, NCT02458196.

Biological agents in the treatment of severe and/ or refractory neuro-Beh(c)et's disease / 中华风湿病学杂志

Objective To explore the use of biological agents in Neuro-Beh(c)et's disease (NBD).Methods We retrospectively reviewed the clinical data of five NBD patients treated with biological agents who were in-patients at Peking Union Medical College Hospital between June 2009 and June 2016.The continuous variables were analyzed by t test.Results All five cases (4 male and 1 female) were severe and/or refractory patients with parenchymal involvement (pNBD).Their age at neurological onset was (31±12) years old.Four cases presented with multiple lesions.The brainstem,spinal cord,cerebral hemisphere and cerebellum involvement were presented in 4,3,3 and 2 patients,respectively.The Rankin score at the onset of NBD was (4.0±0.7).The biological agents were administrated when corticosteroids and immunosuppressant were ineffective.Three cases received tumor necrosis factor (TNF)-α inhibitors therapy,among whom one patient had gastrointestinal ulceration.One patient with refractory retinal vasculitis received interferon-α therapy.One patient treated with tocilizumab [interleukin (IL)-6R inhibitor] had high level IL-6 in the cerebrospinal fluid.All patients achievcd clinical improvements and the Rankin score significantly decreased to (2.2±0.8) when compared with the baseline (t=4.81,P＜0.01) after the treatment with biological agents.The corticosteroid dose was tapered in all cases and the numbers of immunosuppressants were reduced in most cases,indicating a potential steroid and immunosup-pressant-sparing effect.No serious adverse events were observed during the follow-up.Conclusion Neurological involvement is a severe complication of Behqet's disease.We can take appropriate biological agents such as TNF-α inhibitors or interferon-α into consideration when patients have severe/refractory pNBD.