mycophenolic-acid and Behcet-Syndrome

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

Frosted branch angiitis is a rare, severe condition. It can be either a primary or a secondary condition and is characterized by rapid deterioration of vision and fulminant retinal vasculitis that manifests as diffuse sheathing of retinal vessels, macular edema, papillitis, vitritis and anterior uveitis. We aimed to describe a case of frosted branch angiitis and cerebral venous sinus thrombosis as an initial neuro-Behçet's disease onset. Diagnosis of Behçet's disease was based on the current 2014 International Criteria for Behçet's Disease and the International consensus recommendation criteria for neuro-Behçet's disease. In addition, a literature review using search parameters of "frosted branch angiitis", "Behçet" and "neuro-Behçet" in the PubMed database is presented.. A 28-year-old Brazilian pardo woman presented to our hospital with abrupt bilateral vision loss associated with recurrent aphthous oral ulcers 6 months before visual symptom onset. A fundus examination showed bilateral widespread retinal vasculitis with venous and arterial white sheathing, optic disc swelling, macular edema, and retinal hemorrhages, leading to the diagnosis of frosted branch angiitis. An extensive systemic workup for retinal vasculitis was uneventful, except for brain magnetic resonance imaging demonstrating cerebral venous sinus thrombosis and lymphocytic aseptic meningitis. A diagnosis of neuro-Behçet's disease was made, and treatment was started with methylprednisolone therapy 1 g/day for 5 consecutive days, followed by oral mycophenolate mofetil and infliximab 5 mg/kg infusion. The patient's response was rapid, with improvement of visual acuity to hand movement and counting fingers by day 7 and final visual acuity of counting fingers and 20/130.. Frosted branch angiitis may be associated with infectious, noninfectious, or idiopathic causes. An extensive workup should be done to exclude systemic vasculitis such as Behçet's disease. Treatment with systemic steroids must be promptly initiated in association with specific treatment aimed at inflammation control and blindness risk reduction.

Topics: Adult; Antibiotics, Antineoplastic; Behcet Syndrome; Female; Fluorescein Angiography; Fundus Oculi; Humans; Magnetic Resonance Imaging; Methylprednisolone; Mycophenolic Acid; Oral Ulcer; Recovery of Function; Retinal Hemorrhage; Retinal Vasculitis; Sinus Thrombosis, Intracranial; Treatment Outcome; Vision Disorders

To determine whether the addition of 26 weeks of subcutaneous peginterferon-α-2b could reduce the requirement for systemic corticosteroids and conventional immunosuppressive medication in patients with Behçet's disease (BD).. We conducted a multicentre randomised trial in patients with BD requiring systemic therapy. Patients were randomised to 26 weeks of peginterferon-α-2b in addition to their standard care or to standard care only and followed 6-monthly for 3 years with BD activity scores and quality of life questionnaires. Patients at one centre had blood taken to measure regulatory T cells (Tregs) and Th17 cells.. 72 patients were included. At months 10-12, while among the entire patient population there was no difference in the corticosteroid dose or immunosuppression use between the treatment groups (adjusted OR 1.04, 95% CI 0.34 to 3.19), post hoc analysis revealed that in patients who were on corticosteroids at baseline the corticosteroid requirement was significantly lower in the peginterferon-α-2b (6.5 (5-15) mg/day) compared with the non-interferon group (10 (8.25-16.5) mg/day, p=0.039). Furthermore, there was a trend towards an improved quality of life that became significant by 36 months (p=0.008). This was associated with a significant rise in Tregs and a decrease in Th17 cells which was still present at 1 year and 6 months after the interferon was stopped. The safety profile was similar with adverse events in 10% in both groups.. The addition of peginterferon-α-2b to the drug regime of BD patients did not significantly reduce their corticosteroid dose required at 1 year. However, in those on corticosteroids at baseline post hoc analysis demonstrated that the addition of peginterferon-α-2b did result in a significant reduction in corticosteroid dose with a significantly improved quality of life and trend to reduce other required immunosuppressive agents. This effect was seen at 1 year and associated with a rise in Tregs suggesting a possible mode for interferon action.. ISRCTN 36354474; EudraCT 2004-004301-18.

Topics: Adrenal Cortex Hormones; Adult; Antiviral Agents; Azathioprine; Behcet Syndrome; Cyclosporine; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Interferon alpha-2; Interferon-alpha; Lymphocyte Count; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Polyethylene Glycols; Quality of Life; Recombinant Proteins; Single-Blind Method; Surveys and Questionnaires; T-Lymphocytes, Regulatory; Tacrolimus; Th17 Cells; Treatment Outcome

Topics: Adult; Behcet Syndrome; Drug Resistance; Enzyme Inhibitors; Female; Humans; Male; Middle Aged; Mycophenolic Acid; Prospective Studies; Tablets, Enteric-Coated; Young Adult

Cyclosporine A and azathioprine are effective on mucocutaneous lesions in Adamantiades-Behçet's disease. Mycophenolate mofetil (MMF) is a drug resembling their activity but with comparably negligible adverse reactions.. A prospective clinical proof-of-principle study was conducted to investigate the effectiveness and toxicity of MMF in mucocutaneous Adamantiades-Behçet's disease.. Thirty patients were to be treated with MMF 2 g/day p.o. for 6 months, in combination with prednisolone 30 mg/day p.o. during the first month of treatment. Inefficacy was followed by an increase in MMF dose to 3 g/day. The primary efficacy variable was the decrease in the disease activity index (DAI) according to a modified variant of the Iran Behçet's Disease Dynamic Activity Measure system.. The study was interrupted due to inefficacy of the compound after the intermediate evaluation of the first 6 patients (aged 37.0 +/- 7.7 years with disease duration of 10.0 +/- 8.9 years) as required by the ethical committee. Although an improvement of the DAI from 5.2 +/- 3.5 to 1.3 +/- 0.5 was found after the first month of combination treatment, withdrawal of prednisolone led to quick relapses with a new index increase (3.0 +/- 3.5). The treatment was discontinued in 3 patients after 3 months, in 2 patients after 4 months and in another one after 5 months due to deterioration of the disease. Introduction of interferon alpha(2a) (3 x 9 million IU 3x/week s.c.) in 3 patients decreased the activity index from 4.0 +/- 1.0 to 0.0 +/- 0.0. No adverse effects were detected under MMF treatment.. MMF (2-3 g/day) is unable to control the signs of mucocutaneous Adamantiades-Behçet's disease.

Topics: Adolescent; Adult; Aged; Anti-Inflammatory Agents; Behcet Syndrome; Child; Drug Therapy, Combination; Female; Follow-Up Studies; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Prednisolone; Treatment Failure

To report the clinical profile of Behcet's disease and its management with immunosuppressants and biologics in a cohort of 25 patients from a tertiary eye care center in South India.. This was a retrospective, observational study. Records of 45 eyes of 25 patients between January 2016 and December 2021 were retrieved from the hospital database. Complete ophthalmic evaluation and systemic examination by the rheumatologist with appropriate investigations had been done. Results were analyzed using Statistical Package for the Social Sciences (SPSS) software.. Males (19, 76%) were found to be more affected than females (6, 24%). Mean age of presentation was 27.68 ± 11.08 years. Twenty patients had bilateral involvement (80%), and unilateral involvement was seen in five patients (20%). Seven eyes of four patients (16%) had isolated anterior uveitis, out of which one patient had unilateral and three patients had bilateral involvement. Twenty-six eyes of 16 patients (64%) had posterior uveitis, out of which six patients had unilateral and 10 had bilateral involvement. Twelve eyes of seven patients (28%) had panuveitis, out of which two patients had unilateral and five had bilateral involvement. Hypopyon was seen in five eyes (11.1%) and posterior synechiae in seven eyes (15.55%). Posterior segment findings included vitritis (24.44%), vasculitis (17.78%), retinitis (17.78%), disc hyperemia (11.11%), and disc pallor (8.89%). Steroids alone were given in five patients (20%) and intravenous methylprednisolone (IVMP) was given in four patients (16%). Immunosuppressive agents along with steroids were given in 20 patients (80%), of which azathioprine alone was given in seven patients (28%), cyclosporin alone was given in two patients (8%), mycophenolate mofetil alone was given in three patients (12%), combination of azathioprine and cyclosporin was given in six patients (24%), and combination of methotrexate and mycophenolate mofetil was given in one patient (4%). Biologics were given in 10 patients (40%) - adalimumab in seven patients (28%) and infliximab in three patients (12%).. Behcet's disease is an uncommon uveitis in India. Addition of immunosuppressants and biologics to conventional steroid therapy gives better visual outcomes.

Topics: Adolescent; Adult; Azathioprine; Behcet Syndrome; Biological Products; Cyclosporins; Female; Humans; Immunosuppressive Agents; Male; Mycophenolic Acid; Retrospective Studies; Steroids; Uveitis; Young Adult

Experience with mycophenolate in uveitis due to Behçet syndrome (BS) is limited. Twelve patients with panuveitis or posterior uveitis who were started mycophenolate were included. Data on demographic characteristics, therapies, ocular attacks, and adverse events were extracted from patient charts. Seven patients with BS uveitis were prescribed mycophenolate for remission induction, of which 6 were refractory/intolerant to conventional immunosuppressives. Mycophenolate was combined with anti-TNFs in 3 patients, resulting in no further ocular attacks. Mycophenolate had to be stopped in the fourth patient due to adverse events. The remaining 3 patients continued to have ocular attacks and were switched to other agents without any drop in visual acuity. Among the 5 patients who were prescribed mycophenolate for maintenance, 2 were relapse free, but 3 experienced ocular attacks. One patient had an exacerbation of mucocutaneous lesions, and 2 experienced adverse events. Mycophenolate monotherapy may not be adequate for remission induction of refractory BS uveitis, but it can be a safe and effective alternative when combined with a biologic agent. It may also be an option for maintenance therapy.

Topics: Behcet Syndrome; Humans; Immunosuppressive Agents; Mycophenolic Acid; Retrospective Studies; Uveitis

Topics: Aged; Anemia, Aplastic; Behcet Syndrome; Female; Humans; Immunosuppressive Agents; Inflammation; Mycophenolic Acid

Topics: Acute Disease; Adalimumab; Administration, Ophthalmic; Administration, Oral; Adolescent; Anti-Inflammatory Agents; Behcet Syndrome; Enzyme Inhibitors; Fluorescein Angiography; Glucocorticoids; Humans; Infusions, Intravenous; Male; Methylprednisolone; Mycophenolic Acid; Ophthalmic Solutions; Photoreceptor Cells, Vertebrate; Prednisolone; Retinal Pigment Epithelium; Tomography, Optical Coherence; Visual Acuity

Topics: Anti-Inflammatory Agents; Behcet Syndrome; Child; Drug Therapy, Combination; Humans; Male; Mycophenolic Acid; Prednisolone; Sinus Thrombosis, Intracranial

We describe a patient with neuro-Behçets disease (NBD) that presented with symptoms of raised intracranial pressure including papilloedema. MRI revealed tumour-like lesions which, on biopsy, confirmed an active vasculitis. Treatment was commenced with prednisone and cyclophosphamide which proved unsuccessful with enlargement of the cerebral mass lesions. Infliximab and mycophenolate were trialled also without benefit. The patient required ventriculoperitoneal shunts to relieve the symptoms of hydrocephalus. Rituximab was then commenced with significant symptomatic and imaging improvement. The case is unique, in our experience, in the need for shunting to relieve the symptoms of hydrocephalus related to vasculitis.

Topics: Adult; Behcet Syndrome; Brain; Cyclophosphamide; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Infliximab; Magnetic Resonance Imaging; Mycophenolic Acid; Papilledema; Prednisone; Rituximab; Treatment Outcome

Topics: Adult; Aneurysm; Behcet Syndrome; Blood Vessel Prosthesis Implantation; Coronary Angiography; Coronary Occlusion; Coronary Vessels; Drug-Eluting Stents; Humans; Male; Mycophenolic Acid; Percutaneous Coronary Intervention; Postoperative Complications; Tomography, Optical Coherence; Ultrasonography, Interventional

A 50-year-old woman with Beçhet's disease presented with episodic diarrhoea and generalised abdominal pain. She was on regular mycophenolate maintenance therapy, being intolerant of both ciclosporin and azathioprine. Previous rectal biopsy was consistent with colitis, probably associated with Beçhet's disease. During this admission, she began passing faecal matter per vaginam. Digital rectal examination confirmed the presence of a large rectovaginal fistula. She underwent urgent laparotomy for a subtotal colectomy with end ileostomy. Although there were no signs of septicaemia preoperatively, small perforations were identified in the caecum and at the splenic flexure on laparotomy. Histopathology confirmed the presence of multiple shallow ulcers throughout the colon with features suggestive of Beçhet's colitis.

Topics: Abdominal Abscess; Abdominal Pain; Behcet Syndrome; Colitis; Colitis, Ulcerative; Colon; Diarrhea; Female; Humans; Ileostomy; Immunosuppressive Agents; Intestinal Perforation; Middle Aged; Mycophenolic Acid; Rectovaginal Fistula

Topics: Anti-Inflammatory Agents; Anti-Inflammatory Agents, Non-Steroidal; Antibodies, Monoclonal; Behcet Syndrome; Cryptococcus neoformans; Humans; Infliximab; Male; Meningitis, Cryptococcal; Middle Aged; Mycophenolic Acid

Topics: Anti-Inflammatory Agents; Azathioprine; Behcet Syndrome; Colonoscopy; Crohn Disease; Humans; Male; Middle Aged; Mycophenolic Acid; Prednisone