mycophenolic-acid and Takayasu-Arteritis

The purpose of this study is to assess the effectiveness of mycophenolate mofetil (MMF) in treating Takayasu arteritis (TA) patients. Embase, Cochrane Library, Pubmed, Clinicaltrials. Gov and three Chinese literature databases (VIP, CNKI, WanFang) were searched; randomized-controlled trials and observational studies that compared the efficacy before and after treatment with MMF were included. The efficacy outcomes were disease activity, the erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) values and steroid dosage. The results were expressed as mean differences with 95% confidence intervals. Compared with the baseline, there were significant reductions in the ESR (-14.92 [25.35, -4.48]), CRP values (-12.99 [-23.29, -2.68]) and the steroid dosage (-17.64 [-24.89, -10.4]) after the addition of MMF, and the disease tended to stabilize. Therefore, MMF might be an alternative immunosuppressive drug for TA for the control of disease activity and to taper the steroid dosage.

Topics: Adolescent; Adult; Biomarkers; Blood Sedimentation; C-Reactive Protein; Chi-Square Distribution; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Male; Mycophenolic Acid; Steroids; Takayasu Arteritis; Treatment Outcome; Young Adult

Vasculitis is more often a disease of women beyond their reproductive years, leaving the challenges of pregnancy management difficult to study. Pregnancy complications, including pregnancy loss and preterm birth, are higher among women with all forms of vasculitis. It seems that controlling the disease before pregnancy may improve the chances of pregnancy success. Many medications used for vasculitis are considered low risk in pregnancy, including prednisone, colchicine, azathioprine, and tumor necrosis factor inhibitors. Cyclophosphamide, methotrexate, and mycophenolate mofetil should be avoided in pregnancy. Controlling disease with low-risk medications may allow women with vasculitis to have the pregnancies they desire.

Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Azathioprine; Behcet Syndrome; Contraindications; Cyclophosphamide; Deprescriptions; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Methotrexate; Mycophenolic Acid; Pregnancy; Pregnancy Complications; Takayasu Arteritis; Tumor Necrosis Factor-alpha; Vasculitis

Mycophenolate mofetil (MMF) has recently been reported as a useful alternative immunosuppressive drug in autoimmune diseases including in Takayasu arteritis (TA). The aim of this study was to verify the efficacy and tolerability of MMF administration in controlling TA disease activity and allowing glucocorticosteroid reduction. Ten consecutive active TA patients followed at the Vasculitis Clinic were enrolled from January 2003 to 2006 and received oral MMF (2 g/day) for an average of 23.3 months. Disease activity assessed using the National Institutes of Health criteria, clinical features, and inflammatory laboratory findings were evaluated. Five patients had received at least one immunosuppressive drug before administration of MMF (four methotrexate, two azathioprine, and one chlorambucil) but had not achieved clinical and laboratory remission. The other five patients received MMF as their first immunosuppressive drug because of an important disease flare during steroid dose reduction. Clinical activity disappeared in all patients with MMF therapy, except in one patient who abandoned the study because of an important headache, attributed to the drug. Moreover, the MMF therapy allowed significant tapering of the prednisone dose in the rest of the nine patients (24.5 +/- 17.1 vs 5.8 +/- 7.8 mg/day; p = 0.0019). Reinforcing this finding, a significant reduction in inflammatory laboratory parameters, erythrocyte sedimentation rate (24.7 +/- 15.5 vs 12.8 +/- 10.8 mm/h; p = 0.036) and C-reactive protein (24.0 +/- 14.9 vs 11.2 +/- 10.7 mg/l; p = 0.0167), was observed. In summary, MMF therapy reduced clinical and laboratory parameters of TA disease activity, suggesting that this drug is a promising immunosuppressive drug, particularly in refractory cases and as a steroid-sparing agent.

Topics: Adolescent; Adrenal Cortex Hormones; Adult; Anti-Inflammatory Agents, Non-Steroidal; Blood Sedimentation; Female; Humans; Immunosuppressive Agents; Inflammation; Male; Mycophenolic Acid; Prospective Studies; Takayasu Arteritis; Time Factors

Topics: Abdominal Pain; Adolescent; Antibiotics, Antineoplastic; Aorta; Carotid Arteries; Computed Tomography Angiography; Diagnosis, Differential; Glucocorticoids; Humans; Male; Mycophenolic Acid; Prednisone; Takayasu Arteritis; Weight Loss

Topics: Adolescent; Aneurysm; Anti-Inflammatory Agents; Antihypertensive Agents; Blood Pressure Determination; Carotid Artery Diseases; Carotid Artery, External; Female; Humans; Magnetic Resonance Angiography; Mycophenolic Acid; Platelet Aggregation Inhibitors; Prednisolone; Takayasu Arteritis; Tomography, X-Ray Computed; Treatment Outcome

To investigate the therapeutic effect of mycophenolate mofetil(MMF) on Chinese Takayasu's arteritis(TAK) patients. Thirty consecutive TAK outpatients were prospectively enrolled during 2013 to 2015. MMF combined with glucocorticoid was the primary treatment regimen. If clinical stable disease could not be reached, another traditional immunosuppressive agent could be added. All patients were evaluated and followed up every 3 months and vascular image studies by Doppler ultrasonography were repeated every 6 months. The effectiveness of MMF was defined as:(1) ESR < 20 mm/hr;(2) CRP < 10 mg/L or hs-CRP<3 mg/L;(3) stable or improved in vascular image studies;(4) clinical assessment is stable, improved or in remission;(5) the dosage of glucocorticoid could be tapered to less than 15 mg/day. ESR < 40 mm/hr, CRP < 20 mg/L or hs-CRP < 6 mg/L, but meet the other three criteria is defined as partial effectiveness. MMF alone combined with corticosteroid was effective in 12(40.0%) patients. When MMF combined with methotrexate less than 15 mg/week, the effective rate was 30.0%(9/30), including partial effective in 3 patients. When MMF combined with azathioprine 100-150 mg/day, the effective rate was 10.0%(3/30), including partial effective in 1 patient. Four patients withdrew due to side effects. Two patients failed to show response. The overall effective rate of therapy including MMF in treating TAK is 80%.

Topics: Adolescent; Adult; Angiography; Biomarkers; Comorbidity; Female; Follow-Up Studies; Humans; Male; Mycophenolic Acid; Neovascularization, Pathologic; Symptom Assessment; Takayasu Arteritis; Treatment Outcome; Young Adult

Takayasu arteritis (TA) in the child remains a therapeutic challenge because corticosteroids and conventional immunosuppressive agents are not always safe or efficacious. The complex formed by interleukin-6 (IL-6) and soluble IL-6 receptor appears to play a pivotal role in the pathogenesis of TA. We describe a favorable response to the anti-IL-6 receptor antibody tocilizumab (TCZ) in a child with aggressive and refractory TA including an assessment of the proinflammatory cytokine profile. A 3-year-old girl with TA consisting of thickening of the aortic arch wall, severe obstruction of the supra-aortic branches, and complete occlusion of both common carotid arteries failed to respond to corticosteroids, methotrexate, tumor necrosis factor α blockade, cyclophosphamide, and mycophenolate mofetil, and 3 years later, the disease remained active with severe manifestations (brain ischemia). The patient underwent percutaneous angioplasty, although significant restenosis was soon documented. After a severe relapse, the patient started TCZ infusions (8 mg/kg for 2 weeks), and a rapid clinical remission was observed, associated with a drastic reduction of inflammatory markers and IL-6 levels. Corticosteroids were withdrawn, the patient's weight and height improved, and bone mineral density values returned to normal. Two years later, TCZ infusions were extended, with no significant side effects. Cerebral ischemia resolved, and recanalization of the previously occluded supra-aortic branches was performed.

Topics: Anti-Inflammatory Agents; Antibodies, Monoclonal, Humanized; Aorta, Thoracic; Arterial Occlusive Diseases; Brachiocephalic Trunk; Brain Ischemia; Carotid Stenosis; Child, Preschool; Drug Resistance; Drug Substitution; Drug Therapy, Combination; Female; Humans; Image Interpretation, Computer-Assisted; Imaging, Three-Dimensional; Immunosuppressive Agents; Inflammation Mediators; Infusions, Intravenous; Magnetic Resonance Angiography; Mycophenolic Acid; Platelet Aggregation Inhibitors; Prednisone; Recurrence; Subclavian Steal Syndrome; Takayasu Arteritis; Ultrasonography, Doppler

Mycophenolate mofetil (MMf) has recently been reported as a useful alternative immunosuppressive drug in autoimmune diseases. There is paucity of literature on its use in Takayasu's arteritis (TA). The aim of this study was to assess the safety and efficacy of MMf in Asian Indian patients with Takayasu's arteritis. Records of 21 consecutive patients with TA on treatment with oral MMF attending our centre from January 2005 to August 2008 were studied. The clinical, laboratory and angiography findings were noted and disease activity assessment was done using Indian Takayasu's arteritis activity score (ITAS) and physician's global observation score at baseline and last follow-up. Eleven patients were on steroids alone at baseline while ten patients had received azathioprine prior to administration of mycophenolate. The mean duration of follow-up on mycophenolate was 9.6 (+/-6.4) months. Nineteen patients (90%) received mycophenolate due to active disease, while in the other two patients, it was given to facilitate steroid tapering. Mycophenolate had to be discontinued in one patient due to skin rash. At the last visit, all the remaining 20 patients who continued mycophenolate had improvement in disease activity as evident by the drop in median ITAS [7 (range 0-19) versus 1 (range 0-7); p=0.001]. A similar trend was noted in laboratory markers of inflammation with a reduction in mean Erythrocyte Sedimentation Rate (ESR) (68+/-36.5 versus 43.2+/-34 mm/first hour; p=0.003) and mean C - Reactive protein (CRP) (31+/-46.7 versus 17.3+/-23.9 mg/L; p=1.00). All patients received concomitant steroids, but there was a significant decrease in steroid dosage from 36 (+/-16) mg/day at baseline to 19 (+/-14) mg/day at last follow-up (p<0.001). This study is the largest series till date establishing the use of mycophenolate as a safe and effective steroid-sparing immunosuppressant in Takayasu's arteritis.

Topics: Adult; Female; Follow-Up Studies; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Retrospective Studies; Takayasu Arteritis; Treatment Outcome; Young Adult

Three children under the age of 3 years presented with malignant hypertension, proteinuria, and acute kidney injury. Takayasu's arteritis was diagnosed on the basis of clinical symptoms of weight loss and low grade fever in conjunction with elevated sedimentation rate and radiographic evidence of aortic and renal artery stenosis. One patient had a renal biopsy which showed arteriolar sclerosis and focal glomerulosclerosis. All three patients required multiple antihypertensive agents, ultimately including angiotensin receptor blockers and/or angiotensin converting enzyme inhibitors. The vasculitis was treated with pulse corticosteroids followed by cyclophosphamide in one patient and mycophenolate mofetil as maintenance therapy in all. Follow-up has ranged from 2 to 8 years. Although global renal function has normalized in each patient, two have unilateral non-function of one kidney. The last patient has persistent aortic and renal artery stenosis with complex collateralization requiring ongoing medical and anticipated surgical management.

Topics: Angiotensin Receptor Antagonists; Angiotensin-Converting Enzyme Inhibitors; Antihypertensive Agents; Biopsy; Child, Preschool; Female; Follow-Up Studies; Glomerulosclerosis, Focal Segmental; Humans; Hypertension, Malignant; Immunosuppressive Agents; Infant; Kidney; Male; Mycophenolic Acid; Renal Artery Obstruction; Takayasu Arteritis; Time Factors; Treatment Outcome

Topics: Adult; Anti-Inflammatory Agents, Non-Steroidal; Coronary Angiography; Female; Humans; Magnetic Resonance Angiography; Mycophenolic Acid; Prednisolone; Takayasu Arteritis

Takayasu arteritis is a rare form of chronic inflammatory disease of the large arterial vessels. Some patients do not respond to steroids or immunosuppressant drugs.. To evaluate the effect of mycophenolate mofetil in patients with severe Takayasu arteritis.. Case series.. Clinical Research Center for Rare Diseases in Bergamo, Italy.. Three patients with Takayasu arteritis.. Mycophenolate mofetil (2 g/d) given orally in two divided doses.. Clinical evaluation and assessment of leukocyte counts were done weekly. Vascular lesions were assessed by using Doppler ultrasonography.. All patients showed clinical benefit, and two resumed work after months of inactivity. Patients were also able to taper and discontinue steroid use. Mycophenolate mofetil was well tolerated, and no signs of toxicity were observed.. Mycophenolate mofetil may be an alternative to steroids and cytotoxic agents in patients with Takayasu arteritis. Before results of controlled trials become available, mycophenolate mofetil should be considered only for patients who do not improve or stabilize with conventional therapy.

Topics: Administration, Oral; Adult; Female; Humans; Immunosuppressive Agents; Leukocyte Count; Middle Aged; Mycophenolic Acid; Takayasu Arteritis; Ultrasonography