mycophenolic-acid and Sarcoidosis

Topics: Adrenal Cortex Hormones; Azathioprine; Enzyme Inhibitors; Humans; Hydroxychloroquine; Immunosuppressive Agents; Methotrexate; Mycophenolic Acid; Sarcoidosis

In general, sarcoidosis treatment should be offered to palliate symptoms and improve quality of life or to prevent end-organ disease. Symptoms include pulmonary as well as extra-pulmonary manifestations of the disease. The assessment of response to disease includes functional studies such as the forced vital capacity. Radiologic imaging such as chest x-ray has also been used to assess response, although standardized measures have rarely been tested. There are sufficient clinical trials to make specific recommendations regarding treatment of symptomatic pulmonary disease. Initial therapy is usually prednisone or a similar glucocorticoid. However, there are several features of this treatment which are unknown. This includes the initial dose, timing of reduction of dose, and when to discontinue treatment. Since many patients are intolerant of prednisone, steroid-sparing alternatives have been studied. Methotrexate is the most widely used anti-metabolite, but azathioprine, leflunomide, and mycophenolate have also been reported as helpful. The biologic agents, especially monoclonal anti-tumor necrosis factor (anti-TNF) antibodies, have proved effective in patients who have failed other treatments. Infliximab, the most widely studied anti-TNF antibody, has proved effective for a range of refractory sarcoidosis. However, there remain questions regarding dose and duration of therapy. For the clinician, the many treatment options allow for a specific treatment regimen for each patient which minimizes risk while enhancing benefit.

Topics: Anti-Inflammatory Agents; Azathioprine; Clinical Trials as Topic; Drug Administration Schedule; Granuloma; Humans; Immunologic Factors; Infliximab; Isoxazoles; Leflunomide; Lung; Methotrexate; Mycophenolic Acid; Prednisone; Respiratory Function Tests; Sarcoidosis; Skin; Tomography, X-Ray Computed

To assess the clinical characteristics, diagnosis, and outcome of cardiac sarcoidosis in a single institution sarcoidosis clinic.. Patients with cardiac sarcoidosis were identified using refined World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) criteria of highly probable and probable. Patient demographics, local and systemic treatments, and clinical outcome were collected.. Of the 1,815 patients evaluated over a 6-year period, 73 patients met the WASOG criteria for cardiac sarcoidosis. The median age at diagnosis was 46 years, with a median follow-up of 8.8 years. Reduced left ventricular ejection fraction (LVEF) was the most common manifestation (54.8%). Patients with arrhythmias experienced ventricular tachycardia or severe heart block, (35.6% and 19.2%, respectively) with or without reduced LVEF. A total of 45 (61.6%) patients underwent cardiac PET scan and/or MRI, with 41 (91.1%) having a positive study. During follow-up, 10 patients (13.7%) either underwent transplant (n = 3) or died (n = 7) from sarcoidosis. Kaplan-Meier survival curves revealed 5- and 10-year survival rates of 95.5% and 93.4%, respectively. Univariate factors of age at diagnosis < 46 years, implantation of pacemaker or defibrillator, mycophenolate treatment, or LVEF > 40% were associated with improved survival. Cox regression analysis demonstrated that age ≥ 46 years and lack of an implanted pacemaker or defibrillator were the only independent predictors of mortality.. The new WASOG criteria were able to characterize cardiac involvement in our sarcoidosis clinic. Age and lack of pacemaker or defibrillator were the significant predictors of mortality for cardiac sarcoidosis, and reduced LVEF < 40% was associated with worse prognosis.. ClinicalTrials.gov; No.: NCT02356445; URL: www.clinicaltrials.gov.

Topics: Adult; Antibiotics, Antineoplastic; Cardiomyopathies; China; Defibrillators, Implantable; Female; Follow-Up Studies; Heart Transplantation; Humans; Kaplan-Meier Estimate; Magnetic Resonance Imaging, Cine; Male; Middle Aged; Mycophenolic Acid; Positron-Emission Tomography; Prognosis; Sarcoidosis; Stroke Volume; Tachycardia, Ventricular; Ventricular Dysfunction, Left

Sarcoidosis is rare in children. Incidence and prevalence of sarcoidosis in India are not known. Renal involvement in childhood sarcoidosis is further rare with no clear data about prevalence. Here we report a case of a 13-year-old girl who presented with sarcoidosis with multi-system involvement including renal sarcoidosis. She initially presented with pyrexia of unknown origin and cervical lymphadenopathy - evaluation of which led to diagnosis of sarcoidosis. Later, after development of pulmonary involvement, she was treated with oral prednisolone and azathioprine. She again defaulted on medicines and later presented with renal failure and was diagnosed with a renal sarcoidosis. She was treated with oral prednisolone and mycophenolate mofetil with which she gradually improved with normal renal functions.

Topics: Adolescent; Azathioprine; Child; Female; Humans; Mycophenolic Acid; Nephritis, Interstitial; Prednisolone; Sarcoidosis

A 45-year-old male with cardiac sarcoidosis verified by cardiac biopsy presented with multiple coexisting arrhythmias, including ventricular tachycardia of more than 1000 episodes per 24 h, paroxysmal atrial fibrillation, and third-degree atrioventricular block. He did not respond to corticosteroids dose of 20-60 mg once daily and mycophenolate mofetil dose of 1 g twice daily for 6 months. Cardiac magnetic resonance (CMR) demonstrated inflammation and late gadolinium enhancement on right ventricular wall and interventricular septum. Positron emission tomography-computed tomography (PET-CT) showed multifocal

Topics: Adalimumab; Arrhythmias, Cardiac; Atrioventricular Block; Cardiomyopathies; Contrast Media; Fluorodeoxyglucose F18; Gadolinium; Humans; Inflammation; Male; Middle Aged; Mycophenolic Acid; Myocarditis; Positron Emission Tomography Computed Tomography; Sarcoidosis

Liver involvement in sarcoidosis may occur in up to 60% of all patients. As many patients experience only minor symptoms, a high number of undiagnosed cases must be assumed. In order to successfully identify patients with hepatic sarcoidosis, a throughout characterization of these patients and their course of disease is necessary.. We collected 40 patients from four German centers to evaluate current treatment standards and course of disease. All of our patients underwent liver biopsy with histologically proven granulomatous hepatitis.. Detailed characterization of our patients showed an overall benign course of disease. Treatment was very diverse with glucocorticoids for 1 year in 55% (22/40), 5-10 years in 18% (7/40), and permanently in 18% (7/40). Other treatments included disease-modifying anti-rheumatic drugs (DMARDs), the conventional non-biological type in 53% of all patients (of these 81% received azathioprine, 46% metotrexate, 10% hydroxychloroquine, 10% mycophenolate mofetil and 10% cyclophosphamide and biologicals in 8%. Despite these very diverse treatments, patients generally showed slow progression of the disease. Two patients died. None of our patients received a liver transplantation.. Patients received diverse treatments and generally showed slow progression of the disease. Based on our experience, we proposed a diagnostic work up and surveillance strategy as a basis for future, prospective register studies.

Topics: Antirheumatic Agents; Azathioprine; Cyclophosphamide; Digestive System Diseases; Humans; Hydroxychloroquine; Mycophenolic Acid; Sarcoidosis

There is a limited evidence base for the treatment of cutaneous sarcoidosis.. To describe treatment modalities and responses in patients with predominantly cutaneous sarcoidosis, in addition to clinical characteristics and prevalence of systemic disease.. Data were prospectively collected over a 6-year period. The Cutaneous Sarcoidosis Activity and Morphology Index was used to assess treatment effectiveness.. In total, 47 patients with biopsy-confirmed cutaneous sarcoidosis were identified. Morphologically, the most common lesions were papules (49%) and plaques (42.6%). The most commonly affected sites were the head and neck (79%); 89.4% had systemic as well as cutaneous disease; 77% received systemic corticosteroid therapy, while 87% required further steroid-sparing treatment; 40% achieved clinical remission with hydroxychloroquine (HCQ) and 88% achieved clinical remission with methotrexate (MTX). OR of achieving remission on MTX compared with HCQ was 9.8 (95% CI 2.4-40.4, P = 0.001). MTX was superior to both azathioprine (AZA) (OR = 22; 95% CI 1.7-285.9; P = 0.02) and mycophenolate mofetil (MMF) (OR = 22; 95% CI 1.7-285.9; P = 0.02) in achieving remission.. HCQ is effective and well-tolerated. MTX was associated with significantly increased probability of achieving clinical remission compared with AZA and MMF.

Topics: Adrenal Cortex Hormones; Adult; Aged; Azathioprine; Clinical Protocols; Dermatologic Agents; Female; Humans; Hydroxychloroquine; Interleukin-12; Interleukin-23; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Phenotype; Prospective Studies; Quinacrine; Referral and Consultation; Remission Induction; Sarcoidosis; Skin Diseases; Tertiary Care Centers; Tumor Necrosis Factor Inhibitors; Young Adult

Cardiac sarcoidosis (CS) is a major cause of morbidity and mortality in patients with systemic sarcoidosis. Steroid-sparing agents are increasingly used, despite a lack of randomized trials or published guidelines to direct treatment.. This retrospective study included 77 patients with CS treated with prednisone monotherapy (n = 32) or a combination with mycophenolate mofetil (n = 45) between 2003 and 2018. Baseline characteristics and clinical outcomes were evaluated. The mean patient age was 53 ± 11 years at CS diagnosis, 66.2% were male, and 35.1% were Black. The total exposure to maximum prednisone dose (initial prednisone dose × days at dose) was lower in the combination therapy group (1440 mg [interquartile range (IQR), 1200-2760 mg] vs 2710 mg [IQR, 1200-5080 mg]; P = .06). On. Mycophenolate mofetil in combination with prednisone for the treatment of CS may minimize corticosteroid exposure and decrease cardiac inflammation without significant adverse effects.

Topics: Adult; Heart Failure; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Prednisone; Retrospective Studies; Sarcoidosis

Clinical manifestations of sarcoidosis vary widely, depending on the intensity of the inflammation and the organ systems affected. So far, no curative treatment exists; the disease can only be suppressed. All treatment options cause side effects affecting quality of life. The aim of this study was to establish and rank the prevalence of self-reported gastrointestinal side effects of drugs used in the treatment of sarcoidosis.. A cross-sectional web-based anonymous survey about complaints and side effects was conducted among sarcoidosis patients in the Netherlands, United Kingdom, and United States of America.. Of the participants, 70% were being treated with one or more drugs. The most important reported side effect was weight gain, associated with increased appetite among prednisone users (as monotherapy as well as in combination with other drugs). Methotrexate (MTX) users especially experienced nausea, with monotherapy as well as combination therapy. Vomiting and weight loss were most prominent among azathioprine and mycophenolate mofetil (MMF) users, whereas diarrhoea was frequently mentioned by MMF and MTX users. The reported side effects of hydroxychloroquine were generally rather mild.. The current study ranked the gastrointestinal side effects associated with pharmacotherapy in sarcoidosis patients. Pharmacotherapy does have multiple gastrointestinal side effects. The strongest association between a reported side effect and drug use was that of weight gain associated with increased appetite among prednisone users. It would therefore be useful for future research to look further into dietary interventions to counter these side effects and reduce their burden.

Topics: Adult; Cross-Sectional Studies; Drug-Related Side Effects and Adverse Reactions; Female; Gastrointestinal Diseases; Humans; Immunosuppressive Agents; Male; Methotrexate; Mycophenolic Acid; Needs Assessment; Prednisone; Quality of Life; Sarcoidosis; Self Report; Weight Gain

Topics: Adalimumab; Aged; Biopsy; Black or African American; Drug Eruptions; Drug Substitution; Exanthema; Humans; Male; Mycophenolic Acid; Sarcoidosis; Skin

In sarcoidosis although no better drug therapy than corticosteroids (CS) has emerged, alternative immunosuppressive agents are used when indicated. Mycophenolate mofetil (MMF) presents rapid action, a considerable safety profile and absence of lung toxicity. Few data exist so far on its use in patients with sarcoidosis. This is a retrospective study on the effectiveness and safety of MMF in patients with sarcoidosis.. All patients with biopsy proven sarcoidosis treated for at least 1 year with MMF from 2008 to 2017 in our department are evaluated.. Eight patients with both pulmonary and extrapulmonary disease are included in the analysis. During follow-up, symptoms and chest radiological findings improved in all. A statistically significant improvement of FEV. MMF as an alternative drug in systemic sarcoidosis, proved safe and effective, permitting the reduction of the dose of oral CS and leading to clinical, functional and radiological improvement.

Topics: Adrenal Cortex Hormones; Aged; Cohort Studies; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Retrospective Studies; Sarcoidosis; Treatment Outcome

To compare the efficacy of methotrexate (MTX) and mycophenolate mofetil (MMF) in the prevention of relapses in neurosarcoidosis.. We conducted a retrospective multicenter study including patients who received MTX or MMF for the treatment of histologically proven neurosarcoidosis. The efficacy of the immunosuppressive drug was assessed by determining the time to relapse.. Forty patients with a diagnosis of neurosarcoidosis (24 men, 16 women, median age at diagnosis 43.5 years) who received at least 3 months of MTX (n = 32) or MMF (n = 14) were included. The immunosuppressive drug was always associated with steroids. The rate of relapse was 47% in the MTX group (0.2 relapses per year of exposure) and 79% in the MMF group (0.6 relapses per year of exposure) (p = 0.058). The median time to relapse was significantly shorter in the MMF group (11 months) compared with the MTX group (28 months) (p = 0.049). Adverse events occurred in 11 patients during MTX therapy and in 1 patient during MMF therapy (p = 0.12).. Relapses of neurosarcoidosis occur frequently, despite the use of an immunosuppressive drug in addition to corticosteroids. MTX significantly increases the survival time without relapse compared to MMF and should be preferred over MMF for the treatment of neurosarcoidosis. This study provides Class IV evidence that for patients with neurosarcoidosis taking steroids, MTX is superior to MMF in reducing the risk of relapse.

Topics: Adolescent; Adrenal Cortex Hormones; Adult; Aged; Central Nervous System Diseases; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Recurrence; Retrospective Studies; Sarcoidosis; Treatment Outcome; Young Adult

Steroids are the mainstay of treatment for renal sarcoidosis. Many patients with sarcoidosis are chronically dependent on steroids and there is limited data on the use of steroid-sparing agents. This is a case of a patient that has remained in remission using mycophenolate mofetil (MMF) as a steroid-sparing agent. The patient is a 56-year-old female with a history of sarcoidosis diagnosed by lymph node biopsy who developed 3 episodes of acute kidney injury (AKI) in the setting of exacerbations of her sarcoidosis, each responding to prednisone treatment. Due to possible lifelong need for prednisone, MMF was started as a steroid-sparing treatment. She tolerated the MMF well and has now been steroidfree for 22 months. There have been only a few case reports about the use of MMF as a steroid-sparing agent in sarcoid-associated renal disease, in which patients could be successfully weaned off steroids. This is the longest reported follow-up of a patient being off steroids while on MMF. It is also notable for the patient having a relapse on the MMF which responded to an increased dose. MMF should be studied further as a potential steroid-sparing agent in the treatment of sarcoid associated renal disease.

Topics: Acute Kidney Injury; Female; Humans; Kidney Diseases; Middle Aged; Mycophenolic Acid; Sarcoidosis

To determine whether patients with juvenile systemic granulomatous disease (JSGD) (Blau syndrome) and uveitis have a characteristic ocular phenotype.. Clinical and imaging data were collected retrospectively from patients attending the Regional Combined Paediatric Rheumatology and Ocular Inflammatory Service, Bristol Eye Hospital. General demographic information, laterality of the uveitis, age at onset, anatomical classification and course of the uveitis, clinical phenotype and specific NOD2 mutation were recorded for each patient.. Seventeen eyes from nine patients (five males; four females) were included in the study. Mean age at the disease onset was 15 months, range 1-84 months. Eight patients had bilateral uveitis. Anterior uveitis was present in five eyes, intermediate uveitis in two eyes, and there were 10 eyes with panuveitis, manifesting as multifocal choroiditis. Appearance of optic disc included indistinct disc margins in six eyes, optic nerve head pallor in six eyes, optic disc vessel sheathing in four eyes, and there was peripapillary hypo/hyperpigmentation in 13 eyes accompanied with characteristic peripapillary nodular excrescences. Among NOD2 mutations, the p.R334W was the most commonly detected (n: four cases), and three patients carried novel variants, the p.E338D and p.D390V variants in one patient, and the p.H520Y and p.Q809K variants in two different patients.. Chronic bilateral panuveitis and a nodular peripapillary appearance in childhood onset uveitis are characteristic features of JSGD, which support the need for an appropriate genetic NOD2 analysis.

Topics: Age of Onset; Arthritis; Child, Preschool; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Infant; Male; Methotrexate; Mutation; Mycophenolic Acid; Nod2 Signaling Adaptor Protein; Optic Nerve; Phenotype; Prednisolone; Retina; Retrospective Studies; Sarcoidosis; Synovitis; Uveitis; Visual Acuity

To describe effectiveness, steroid-sparing effect, and tolerance of the antiproliferative immunosuppressant mycophenolate mofetil (MMF) in neurosarcoidosis.. We describe a retrospective case series of 10 consecutive patients with a diagnosis of neurosarcoidosis who were treated with MMF, alone or in association with corticosteroids, in our teaching hospital.. At the time of our study, the mean duration of MMF treatment was 21 months. All but one patient with CNS involvement (n = 8) were in remission (except for hormonal dysfunction) which was complete in 6 patients. MMF was efficient as single-agent induction therapy in one patient. The 3 patients who received MMF as a maintenance therapy after initial response to corticosteroids did not relapse even though steroids were stopped. Out of 4 subjects who demonstrated insufficient response to prior therapy including corticosteroids and immunosuppressive agents, 3 demonstrated significant clinical and radiologic improvement. However, the 2 patients who presented muscular sarcoidosis did not respond to MMF. Among patients treated with steroids at MMF introduction and after excluding those with sarcoid myopathy, the mean dose of corticosteroids was 6 mg/day at the end of the follow-up while it was 59 mg/day at the initiation of MMF. No significant side effects were observed.. These data suggest that MMF is effective in CNS sarcoidosis but not in sarcoid myopathy, with a corticosteroid sparing effect and a better tolerance profile than other immunosuppressive agents.

Topics: Adult; Aged; Central Nervous System Diseases; Female; Humans; Immunosuppressive Agents; Male; Muscular Diseases; Mycophenolic Acid; Retrospective Studies; Sarcoidosis; Treatment Outcome

Spinal cord involvement in sarcoidosis is rare, occurring in <1% of patients with sarcoidosis.. We report seven cases of spinal cord sarcoidosis, seen in two French hospitals over a 13-year period. Presentation of disease, methods of diagnosis and response to treatment, with quantification according to the reduction of the modified Rankin scale (MRS), were noted.. Six patients presented insidious paresthesias or weakness and one a sudden paraplegia. Average MRS at diagnosis was to 2. Spine MRI showed one or several intramedullary lesions in all cases. Diagnosis was confirmed by extra-neural tissue biopsies in all cases, including mediastinoscopy (two patients), coelioscopy (one patient), bronchoscopy (one patient), salivary gland biopsy (one patient) and skin biopsy (two patients). The average follow-up for the group was 49.4 months. All patients responded to corticosteroid therapy with a median reduction of MRS of one point. Five patients received immunosuppressive therapy: cyclophosphamide (two patients), methotrexate (two patients), azathioprine (one patient), mycophenolate mofetyl (one patient), with an inconstant benefit. Patients who received cyclophosphamide presented severe fungaemia.. Based on our study and literature analysis, we propose an algorithm for treatment of spinal cord sarcoidosis.

Topics: Adrenal Cortex Hormones; Adult; Aged; Azathioprine; Cyclophosphamide; Female; Fungemia; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Sarcoidosis; Severity of Illness Index; Spinal Cord; Spinal Cord Diseases

To describe the effects of the anti-tumor necrosis factor neutralizing antibody, infliximab, and the antiproliferative immunosuppressant, mycophenolate mofetil, in refractory neurosarcoidosis.. We treated patients with biopsy-proven sarcoidosis and CNS involvement, who had failed treatment with steroids, with infliximab (5 mg/kg on weeks 0, 2, and 6, and then every 6-8 weeks thereafter). Six out of seven patients were co-treated with mycophenolate mofetil (1,000 mg PO BID). Patients underwent a review of symptoms and complete neurologic examination every 3 months and MRI scanning before and after 3-4 infusions of infliximab.. All patients reported significant symptomatic improvement by the fourth infusion of infliximab, including relief of headache and neuropathic pain, reversal of motor, sensory, or coordination deficits, and control of seizure activity. Furthermore, infliximab therapy was universally associated with a decrease in lesion size or suppression of gadolinium enhancement as documented by MRI. A positive treatment response was attained irrespective of location or distribution of CNS involvement by sarcoidosis (dural/leptomeningeal based vs intraparenchymal; cord vs brain; single lesion vs multifocal). There were no serious adverse effects in a follow-up period spanning 6-18 months.. Combination treatment with mycophenolate mofetil and infliximab is a promising therapeutic approach for neurosarcoidosis.

Topics: Adult; Antibodies, Monoclonal; Central Nervous System Diseases; Drug Therapy, Combination; Female; Follow-Up Studies; Humans; Infliximab; Male; Middle Aged; Mycophenolic Acid; Sarcoidosis; Treatment Outcome

Sarcoidosis is a multisystem granulomatous disease that may involve the bone marrow, with resultant fever, anemia, and leukopenia. Although generally effective in treating the clinical manifestations of bone marrow sarcoidosis, systemic corticosteroids are not warranted for long-term therapy because of their well-known adverse effects. Therefore, alternative corticosteroid-sparing therapeutic regimens are desired.. A 41-year-old man sought treatment for cutaneous and bone marrow sarcoidosis resulting in fatigue, anemia, and leukopenia refractory to conventional therapies and mycophenolate mofetil. We initiated combination immunosuppressive therapy with methotrexate sodium and mycophenolate mofetil, which resulted in a safe and prolonged quiescence of cutaneous disease and resolution of anemia and leukopenia throughout a 34-month follow-up period.. We present this case to highlight the growing body of evidence supporting combination immunosuppressive therapy to treat refractory sarcoidosis. In our patient, sarcoidal bone marrow involvement responded dramatically to a combined regimen of methotrexate and mycophenolate mofetil with no significant adverse effects, despite previously having been refractory to conventional agents and mycophenolate mofetil alone. This report provides evidence that combination immunosuppressive therapy is a potential treatment of refractory bone marrow sarcoidosis and highlights important issues about combined immunosuppressive therapy.

Topics: Adult; Anemia; Bone Marrow Diseases; Drug Therapy, Combination; Humans; Immunosuppressive Agents; Leukopenia; Male; Methotrexate; Mycophenolic Acid; Sarcoidosis; Skin Diseases

To evaluate the association between scleritis and systemic disease in a non-university, non-tertiary referral practice and to describe our experience with scleritis treatment.. Retrospective chart review.. The medical records of patients with scleritis between 2001 and 2007 were reviewed for associated systemic disease.. In our series of 86 patients with scleritis, 55 patients (64.0%) had isolated scleritis while 31 patients (36.0%) had associated systemic-disease. Twenty-six patients (83.9%) with systemic disease had diagnosed systemic disease at the time of initial scleritis presentation, while 5 patients (16.1%) were diagnosed following systemic work-up. Those diagnosed after systemic work-up were more likely to have systemic vasculitic disease as opposed to a rheumatic or infectious disease. Patients with and without associated systemic disease were likely to require systemic therapy at similar rates (93.5% and 92.7%, respectively). Five patients with steroid-refractory scleritis were treated with infliximab (Remicade; Centocor Inc, Horsham, Pennsylvania, USA) and all responded without evidence of adverse effect. Seven patients were treated with mycophenolate mofetil (CellCept; Roche Laboratories, Nutley, New Jersey, USA), of which three improved.. The association between scleritis and systemic disease in a community-based referral practice may be lower than in tertiary referral or university-based centers. Although thorough systemic disease evaluation is warranted in scleritis patients, most patients with associated systemic disease will have such a diagnosis prior to the development of scleritis. The need to institute aggressive systemic therapy cannot be predicted by the presence of an associated systemic disease. Infliximab and mycophenolate mofetil are useful additions to the scleritis practitioner's armamentarium for steroid-refractory scleritis.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents; Antibodies, Monoclonal; Autoimmune Diseases; Child; Community Health Services; Female; Herpes Zoster; HIV Infections; Humans; Infliximab; Male; Middle Aged; Mycophenolic Acid; Referral and Consultation; Retrospective Studies; Sarcoidosis; Scleritis; Young Adult

Neurosarcoidosis is a rare (5 cases for one million) immune-mediated disease generally observed in young adults. Neurological symptoms are present in the half of patients, and symptoms remain limited to neurological system in 10p.cent. Histological criteria are mandatory to prove the diagnosis. The sensitivity and complications of biopsy are variable. The best sensitivity appears to be achieved with muscle biopsies which in addition have a lower risk of complications. Neurosarcoidosis is usually treated with corticosteroid therapy and immunosuppressive drugs (cyclophosphamide, cyclosporine, aziathoprine, methotrexate), but frequently resists standard schedules. In addition the many contraindications, side effects and cumulated toxicities of immunosuppressive drugs compromises their use. Knowledge of the effectiveness of other treatments would therefore be useful. Mycophenolate mofetil (MMF) has been used for treatment of many immune-mediated neurological diseases, like polymyositis, multifocal motor neuropathy, myasthenia or chronic inflammatory demyelinating polyradiculoneuropathy. MMF is efficient and well tolerated, but there is no case-report about neurosarcoidosis.. We report two observations of young patients (14 and 27 years) with a diagnosis of resistant neurosarcoidosis treated with MMF (2 g/j) and corticosteroids. A significant and rapid effectiveness was clinically and radiologically observed, with good clinical and hematologic tolerance.. The MMF seems to be an interesting rescue treatment for neurosarcoidosis. Further evaluation is needed.

Topics: Adolescent; Adrenal Cortex Hormones; Adult; Anti-Inflammatory Agents; Anti-Inflammatory Agents, Non-Steroidal; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Mycophenolic Acid; Peripheral Nervous System Diseases; Sarcoidosis

Sarcoidosis is a multi-system disorder characterized by non-caseating epithelioid granulomas in multiple organs. The disease usually presents in young adults and is uncommon in children. Renal involvement can usually occur due to granulomatous interstitial nephritis, but renal failure is uncommon. Corticosteroids are the mainstay of therapy. We present the report of a child with severe renal failure secondary to renal limited sarcoidosis who was successfully treated with corticosteroid induction therapy. Because of the severe side effects of corticosteroids, mycophenolate mofetil was added and corticosteroids were tapered off. The child has been in sustained remission for over a year with mycophenolate mofetil monotherapy.

Topics: Adolescent; Adrenal Cortex Hormones; Humans; Immunosuppressive Agents; Kidney Diseases; Male; Mycophenolic Acid; Remission Induction; Sarcoidosis

Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.

Topics: Child; Dose-Response Relationship, Drug; Glucocorticoids; Heart Transplantation; Humans; Immunosuppressive Agents; Lymph Nodes; Male; Mycophenolic Acid; Postoperative Complications; Prednisolone; Radiography, Thoracic; Recurrence; Retreatment; Sarcoidosis; Tacrolimus; Tomography, X-Ray Computed

Sarcoidosis is an inflammatory disease with potentially severe mucocutaneous manifestations. Mycophenolate mofetil (MMF) is an immunosuppressive drug extensively used in organ transplantation. Its use has been rapidly expanded into autoimmune and inflammatory diseases. We report the first successful and safe use of MMF in five patients with sarcoidosis.

Topics: Adult; Dermatologic Agents; Drug Resistance; Female; Follow-Up Studies; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Sarcoidosis; Skin Diseases

Topics: Humans; Immunosuppressive Agents; Mycophenolic Acid; Sarcoidosis; Skin Diseases; Uveitis

Topics: Anti-Inflammatory Agents, Non-Steroidal; Duodenitis; Humans; Male; Middle Aged; Mycophenolic Acid; Pancreatitis; Sarcoidosis