Is Intravenous Immunoglobulin Research Articles

Fresh frozen plasma (FFP) for hypogammaglobulinemia (HypoG) in patients (Pts) with recurrent infections (Infxs) after B-cell directed treatment: A single institution experience with economic impact analysis.

e13519 Background: The incidence of hypoG with recurrent infxs has rapidly increased in pts undergoing B-cell targeted therapies, including monoclonal and bispecific antibodies and chimeric antigen receptor (CAR) T-cell therapies, for benign and malignant diseases. These novel therapies produce life-threatening hypoG due to persistently severe depletion of B cells. The standard of care (SoC) for hypoG is intravenous immunoglobulin (IVIG) infusion, which is costly with limited efficacy. FFP is known to have B cell-related immunologic elements (IgM, A, & D) and complement that are essential for fighting infx. Methods: We retrospectively identified pts between January 2020 and 2024 diagnosed with hypoG who received FFP or IVIG for recurrent infx from B-cell targeted therapies (67% received anti-CD20 agents). Data was collected 12 months (mos) before and after FFP or IVIG infusion. HypoG-related recurrent infx are usually chronic with no documented positive cultures. Therefore, we reviewed each pt’s subjective reports of infectious improvement. Our primary endpoint was the annualized infx rate before and after FFP or IVIG, with a secondary outcome comparing hospitalization rates. Infxs were measured by courses of antimicrobial therapy. A Kaplan-Meier analysis was performed with accompanying log-rank testing for the primary outcome. Results: 21 pts met inclusion criteria, 5 received FFP and 16 received IVIG. Median (mdn) age was 72 years (range, 46 - 87) and 57.1% were female. 66.6% received FFP/IVIG after therapy for hematologic cancers (62% B cell lymphoma), 28.6% for solid tumors, and 4.8% for autoimmune disorders. The mdn number of infxs in the 12 mos preceding FFP and IVIG were 3 (2 - 5) and 3 (0 - 13), respectively, while the mdn number of infx-related hospitalizations prior to intervention was 2 (1 - 3) and 0 (0 - 6). The mdn number of infxs in the 12-mo period following FFP initiation was 1 (0 - 2) versus 0 (0 - 3) after IVIG initiation, and mdn number of infx-related hospitalizations post-intervention was 0 (0 - 1) and 2 (0 - 7). The mdn time to first infx on FFP and IVIG were 5 mos (2 - 12) and 4 mos (1 - 12), respectively, with one pt in each cohort not having an outcome. All 5 pts in the FFP cohort had documented subjective improvement of infectious symptoms after first infusion, as opposed to none in the IVIG cohort (p = 0.000). There was no significant difference in mdn time to first infx (p = 0.591). Conclusions: IVIG costs about 10,000 USD per infusion compared to 120 USD for FFP, excluding labor, distribution and facility charges. The estimated total IVIG market share in the US is about 5 billion in 2024. Replacement of IVIG with FFP will alleviate supply constraints and save US healthcare at least 4.95 billion USD yearly. Comparable rates of infx with FFP versus IVIG suggest that FFP is non inferior to the SoC for treating hypoG.

ОПЫТ ПРИМЕНЕНИЯ ИНФЛИКСИМАБА В ЛЕЧЕНИИ ИММУНОГЛОБУЛИН-РЕЗИСТЕНТНОЙ БОЛЕЗНИ КАВАСАКИ

Kawasaki disease (KD) is an acute systemic disease characterized by predominant damage to small and medium-sized arteries with the development of destructive-proliferative vasculitis, damage to the coronary arteries (CA) and other visceral arteries manifested by fever, changes in the mucous membranes, skin, and lymph nodes. The initial KD therapy is intravenous immunoglobulin (IVIG) at a dose of 2 g/kg coupled with acetylsalicylic acid. However, 10% to 38% may be resistant to standard therapy. There are no uniform recommendations for the management of patients with immunoglobulin-resistant KD. Possible therapies include repeated IVIG, high-dose glucocorticoids, genetically engineered biological drugs and other cytostatic drugs. Authors represent a clinical case of a 4-month-old boy with a complete form of KD, giant CA aneurysms and a resistance to the previous 4 courses of immunomodulatory therapy (2 courses of IVIG, etanercept, pulse therapy with methylprednisolone) with the effective use of the tumor necrosis factor alpha blocker, infliximab.

Five Years Follow-up of Opsoclonus-Myoclonus-Ataxia Syndrome-Associated Neurogenic Tumors in Children.

Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare autoimmune disorder. Approximately half of the cases are associated with neuroblastoma in children. This study's aim is to review management of our cases with OMAS-associated neuroblastoma for treatment approach as well as long-term follow-up. Age at onset of symptoms and tumor diagnosis, tumor location, histopathology, stage, chemotherapy, OMAS protocol, surgery, and follow-up period were evaluated retrospectively in six patients between 2007 and 2022. Mean age of onset of OMAS findings was 13.5 months and mean age at tumor diagnosis was 15.1 months. Tumor was located at thorax in three patients and surrenal in others. Four patients underwent primary surgery. Histopathological diagnosis was ganglioneuroblastoma in three, neuroblastoma in two, and undifferentiated neuroblastoma in one. One patient was considered as stage 1 and rest of them as stage 2. Chemotherapy was provided in five cases. The OMAS protocol was applied to five patients. Our protocol is intravenous immunoglobulin (IVIG) 1 g/kg/d for 2 consecutive days once a month and dexamethasone for 5 days (20 mg/m2/d for 1-2 days, 10 mg/m2/d for 3-4 days, and 5 mg/m2/d for the fifth day) once a month, alternatively by 2-week intervals. Patients were followed up for a mean of 8.1 years. Neuropsychiatric sequelae were detected in two patients. In tumor-related cases, alternating use of corticosteroid and IVIG for suppression of autoimmunity as the OMAS protocol, total excision of the tumor as soon as possible, and chemotherapeutics in selected patients seem to be related to resolution of acute problems, long-term sequelae, and severity.

MYASTHENIA GRAVIS

Myasthenia gravis is an autoimmune disease of the postsynaptic membrane, especially acetylcholine receptors in the neuromuscular link of skeletal muscle. Patients with myasthenia gravis have a high number globally. The disease occurs due to a disorder that impairs the impulse connection between chemicals traveling from nerve endings and receptors. Clinical symptoms include weakness of the eye muscles (ptosis and diplopia), difficulty swallowing, and difficulty speaking. The diagnosis of myasthenia gravis is based on the patient's complaints obtained in the history, physical and neurological examination, and supporting examinations. The management that can be given is intravenous immunoglobulin (IVIg) therapy, plasma exchange (PE), corticosteroids given together with IVIg and PE, or acetylcholinesterase inhibitors. These treatments can determine the patient's prognosis. If the patient with myasthenia gravis is left to involve the respiratory muscles, then the patient's prognosis becomes worse. In addition, myasthenic crisis and cholinergic crisis may occur, which is a medical emergency.

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Abstract 10405: Associations Between Ivig Dose and Outcome of Kawasaki Disease Patients in Acute Phase

Background: The standard treatment of Kawasaki Disease (KD) is intravenous immunoglobulin (IVIG) 2g/kg and aspirin. However, the most effective dosage of IVIG to prevent coronary artery abnormalities (CAA) is not known. The purpose of this study was to identify the dose of IVIG for KD patients in acute phase using a national inpatient database in Japan. Method: We used the Diagnostic Procedure Combination database to identify KD patients treated with IVIG between 2010 and 2017. We used multiple logistic regression models to estimate the risk of proportion of CAA and IVIG resistance, and multiple regression models to estimate the difference of length of stay and medical cost. In all models, demographic characteristics and comorbidities were adjusted for as covariates. We generated restricted cubic splines models with the same covariate specification as the fully adjusted model with five knots to examine non-linear associations between IVIG dose and the different outcomes. Results: We extracted 42,331 eligible patients from the database. The age was 1.7 years (1.6 SD), male 24432 (58%), and body weight 11.1kg (4.9 SD). The mean dose of IVIG was 1.7g/kg (1.3 SD), proportion of CAA 1325(3.1%), and medical cost 6596 USD (5160- 8689). We found a reverse J-shaped association between the dose of IVIG and the proportion of CAA. IVIG dosage was also found to have U-shaped associations with length of stay and medical costs. Conclusion: When IVIG dose was less than 2g/kg, the proportion of CAA increased, and the length of stay and medical cost also increased; when IVIG dose was more than 2g/kg, the length of stay and medical cost could be increased, and 2g/kg IVIG was considered appropriate for the initial treatment of KD.

Efficacy of the Delayed Use of Low-dose Aspirin in Intravenous Immunoglobulin Therapy for Acute-phase Kawasaki Disease

The mainstay of current standard therapy for acute-phase Kawasaki disease (KD) is intravenous immunoglobulin (IVIG) therapy at 2 g/kg. However, the efficacy of combining medium- or high-dose aspirin with IVIG therapy at 2 g/kg has not been fully investigated. Some studies suggested that aspirin may inhibit coronary artery lesion (CAL) prevention in IVIG therapy and that the delayed use of aspirin in IVIG therapy may be beneficial for the suppression of CALs and prevention of coronary artery stenosis in patients with KD. The efficacy of the delayed use of low-dose aspirin in IVIG therapy for acute-phase KD remains unclear. Therefore, this retrospective study aimed to assess the efficacy of the delayed use of low-dose aspirin, when combined with IVIG therapy for acute-phase KD. Data were obtained from 193 KD patients who underwent acute-phase treatment from January 2009 to October 2020 and IVIG therapy at 2 g/kg with the delayed use of aspirin/flurbiprofen. The patients were divided into three groups: (1) low-dose group, in which 40 patients received low-dose aspirin (5 mg/kg/day); (2) medium-dose group, in which 90 patients received medium-dose aspirin (30 mg/kg/day); and (3) flurbiprofen group, in which 63 patients received flurbiprofen (3–5 mg/kg/day). KD patients with liver damage or those present during influenza season underwent flurbiprofen therapy between January 2009 and November 2017. All patients except one received low-dose aspirin after December 2017. The serum albumin level (median 3.40 vs. 3.30 g/dL, P = 0.026) and Egami score (median 1.0 vs. 2.0, P < 0.001) before the initial treatment were significantly different between the medium-dose group and the flurbiprofen group. The rates of initial IVIG therapy resistance (25.0% vs. 18.9% vs. 25.4%, P = 0.790), rescue therapy (17.5% vs. 8.9% vs. 17.5%, P = 0.721), and CALs (5.0% vs. 0.0% vs. 4.8%, P = 0.713) were similar among the low-dose, medium-dose, and flurbiprofen groups. Overall, the efficacy of the delayed use of low-dose aspirin was similar to that of the delayed use of medium-dose aspirin/flurbiprofen in IVIG therapy for acute-phase KD.

A systematic review of the various treatment options regarding the effectiveness of IVIG for nephropathy due to BK virus

"Abstract: Introduction: BK virus is an opportunistic infectious disease that causes disease and serious problems when the immune system is suppressed. One of the treatments used against this virus is intravenous immunoglobulin (IVIG). We aimed to review the major relevant articles in case of the efficacy of IVIG and determine its usefulness. Methods: We searched online databases such as PubMed, MEDLINE, Wiley, EMBASE, ProQuest Dissertations and Thesis, ISI Web of Knowledge, Scopus, and Google scholar. Two reviewers have independently assessed and extracted the titles and abstracts. Disagreements were being fixed by discussion. Where resolve was not feasible, a third review author was discussed. Results: We screened a total of 6 full texts. Three studies evaluated the effectiveness of IVIG in the Treatment of BK Infection in Renal Transplant Patients. Also, three studies assessed the various treatment options for Nephropathy due to the BK virus. Results showed that mean peak BK reduced with IVIG therapy after a one-year follow-up. Also, a high percentage of patients have functioning grafts after IVIG therapy. Conclusion: A review of studies shown powerful follow-up and early decrease of immunosuppression leading detection of BK viremia, with qualitative monitoring, can avoid the progress of clinically notable BK nephropathy. Combination treatment IVIG is more successful in removing viral load in patients with BKVAN, compared with traditional standard-ofcare therapy. Keywords: BK virus, nephropathy, treatment"

Neuroinflammation after SCI: Current Insights and Therapeutic Potential of Intravenous Immunoglobulin.

Traumatic spinal cord injury (SCI) elicits a complex cascade of cellular and molecular inflammatory events. Although certain aspects of the inflammatory response are essential to wound healing and repair, post-SCI inflammation is, on balance, thought to be detrimental to recovery by causing "bystander damage" and the spread of pathology into spared but vulnerable regions of the spinal cord. Much of the research to date has therefore focused on understanding the inflammatory drivers of secondary tissue loss after SCI, to define therapeutic targets and positively modulate this response. Numerous experimental studies have demonstrated that modulation of the inflammatory response to SCI can indeed lead to significant neuroprotection and improved recovery. However, it is now also recognized that broadscale immunosuppression is not necessarily beneficial and may even carry the risk of contributing to the development of serious adverse events. Immune modulation rather than suppression is therefore now considered a more promising approach to target harmful post-traumatic inflammation following a major neurotraumatic event such as SCI. One promising immunomodulatory agent is intravenous immunoglobulin (IVIG), a plasma product that contains mostly immunoglobulin G (IgG) from thousands of healthy donors. IVIG is currently already widely used to treat a range of autoimmune diseases, but recent studies have found that it also holds great promise for treating acute neurological conditions, including SCI. This review provides an overview of the inflammatory response to SCI, immunomodulatory approaches that are currently in clinical trials, proposed mechanisms of action for IVIG therapy, and the putative relevance of these in the context of neurotraumatic events.

Aseptic Meningitis, As the First Manifestation of Kawasaki Disease: A Case Report

: Kawasaki disease (KD), a systemic inflammatory disorder with medium-sized vasculitis, mostly occurs among children < five years of age. The highest incidence of KD is among the Asian population. The primary treatment of KD is intravenous immunoglobulin (IVIG) administration. Extreme irritability is a common neurologic manifestation among infants, and central nervous system involvement is uncommon and occurs as a result of vasculitis or IVIG administration late in the treatment course. Here, we discussed an eight-year-old girl with a final diagnosis of atypical KD, who was primarily diagnosed as aseptic meningitis. The patient was admitted with a history of fever, headache, and vomiting and later developed strawberry tongue, coronary involvement, and fingertip desquamation during the follow-up period. Aseptic meningitis, as the first clinical manifestation, made the definitive diagnosis of the KD challenging.

Frequently asked questions regarding treatment of Kawasaki disease.

[first paragraph of article]The mainstay of therapy for acute Kawasaki disease (KD) is intravenous immunoglobulin (IVIG), which was first described in a case series from Japan and later proven through a nationwide clinical trial in the U.S. published in 1986. Since completion of the initial clinical trials, many questions have arisen regarding the nuances of KD treatment. In the absence of an evidence base, what follows is an attempt to devise rational responses to these questions that draw upon common sense and the personal experience of this author.

Management of pemphigus disease in pregnancy.

Pemphigus can cause complications during pregnancy and may cause serious harm to a fetus. For this study, a comprehensive review of common treatments of pemphigus and their adverse effects associated with pregnancy and male fertility was conducted. We concluded that a period of remission with minimal or no therapy before conception could significantly reduce the risk of the disease flaring up, at least in the first trimester. The period of remission causes a delay in the flare-up of the disease, which means lower cumulative doses and the prevention of possible congenital abnormalities caused by corticosteroid or immunosuppressant treatments. All common treatments of pemphigus-azathioprine, mycophenolate mofetil, and methotrexate-should be avoided during pregnancy. However, it appears that systemic corticosteroids in a safe dose with a topical form of corticosteroids may be used without serious risk. Due to the lack of data associated with rituximab therapy, it is recommended that this drug be avoided 12months before conception. It appears that the safest treatment of pemphigus is intravenous immunoglobulin (IVIg), which may be more effective when used with topical corticosteroids. Due to the delayed effect of IVIg, it should be used some months prior to conception.

Mechanistic properties of intravenous immunoglobulin in murine immune thrombocytopenia: support for FcγRIIB falls by the wayside

Immune thrombocytopenia (ITP) is an autoimmune disorder characterised by platelet clearance resulting from the production of platelet-reactive autoantibodies. Platelet clearance appears to occur mainly via phagocytosis in the mononuclear phagocytic system, although T-cell–mediated platelet destruction, platelet apoptosis and dysregulation of platelet production can also play a role in disease pathogenesis. One of the most successful treatments for ITP is intravenous immunoglobulin (IVIg), and while it has been used in ITP for over 30 years, its mechanism(s) of action still remain unclear. Animal models of ITP have proven useful in understanding IVIg’s immunomodulatory properties, providing a valuable tool to test new mechanistic theories as well as further explore the soundness of older ones. This model has also provided the key evidence that IVIg exerts its effects via activating receptors for IgG Fc, specifically FcγRIII, via formation of IgG dimers or immune complexes. Here, we discuss the validity of one prominent theory of IVIg function, anti-inflammatory activity mediated through the inhibitory Fcγ receptor FcγRIIB, and review evidence to suggest that this theory is not likely valid in the practical sense.

Sa1113 Is Intravenous Immunoglobulin (IVIG) Therapy Beneficial in Reducing Utilizations of Healthcare Resources in Patients With Drug and Device Refractory Symptoms of Gastroparesis?

Sa1113 Is Intravenous Immunoglobulin (IVIG) Therapy Beneficial in Reducing Utilizations of Healthcare Resources in Patients With Drug and Device Refractory Symptoms of Gastroparesis?

Kawasaki disease: a comprehensive review of treatment options.

Kawasaki disease (KD) is an acute self-limiting systemic vasculitis with specific predilection for the coronary arteries that affects previously healthy young infants and children. It is the leading cause of childhood-acquired heart disease in the developed world. Although the stimulus for the cascade of inflammation in KD is unknown, prompt treatment within 10 days of symptom onset has been shown to improve clinical outcomes and reduce the risk of coronary artery complications. Standard initial therapy is intravenous immunoglobulin (IVIG) and aspirin. Non-responders to initial therapy remain a challenge. This present review summarizes the treatment options for initial and refractory KD, including the role of steroids and other immunosuppressive therapies. Literature search using PubMed database to identify pharmacologic studies in KD using the terms Kawasaki disease, intravenous immunoglobulin, refractory, corticosteroids, infliximab, cyclosporine, methotrexate, high risk from January 1988-May 2015 was performed. Bibliographies of selected references were also evaluated for relevant articles. Results were limited to those published in English. All articles identified from the PubMed searches were evaluated. Initial IVIG therapy results in rapid resolution of clinical symptoms in 80-90% of patients and has been shown to reduce the risk of coronary disease. Although concomitant aspirin remains the standard of care for the initial management of KD, the evidence to support its efficacy in improving coronary artery outcomes are lacking. Initial therapy with corticosteroids in addition to intravenous immunoglobulin and aspirin improves outcomes in patients in Japan. However, identifying patients at high risk who may benefit from additional corticosteroids in heterogeneous populations has been challenging. Therapeutic options for non-responders to initial therapy are also challenging given the paucity of data. Patients who fail to respond to the first dose of IVIG will most often receive a second dose. Patients who fail to respond to two doses of IVIG present a unique challenge as the appropriate treatment remains uncertain. Although their effectiveness remains unproven, treatment with infliximab, cyclosporine or methotrexate may be considered in those patients who fail multiple doses of IVIG and steroids. The role of steroids in high-risk non-Japanese patients is unclear, with the biggest challenge being early identification of patients at high risk of developing adverse coronary artery outcomes. Limited data evaluating other immunosuppressive agents are available and should be reserved for patients failing two doses of IVIG. Although recent advances in research have broadened our understanding of the epidemiology, genetic susceptibility and pathogenesis of KD, the aetiology of KD remains unclear. Ongoing research will help determine more precise pathogenesis and may assist in developing a diagnostic test as well as identifying new targets for more precise treatment interventions.

Management of Kawasaki disease in resource-limited settings.

Management of Kawasaki disease in resource-limited settings.

New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin

Common variable immune deficiency (CVID) is the most frequent symptomatic primary immune deficiency in adults. The standard of care is intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (scIG) therapy. The cause of CVID is currently unknown, and there is no universally accepted definition of CVID. This creates problems in determining which patients will benefit from IVIG/scIG treatment. In this paper, we review the difficulties with the commonly used European Society of Immune Deficiencies (ESID) and the Pan American Group for Immune Deficiency (PAGID) definition of CVID. We propose new criteria for the diagnosis of CVID, which are based on recent scientific discoveries. Improved diagnostic precision will assist with treatment decisions including IVIG/scIG replacement. We suggest that asymptomatic patients with mild hypogammaglobulinaemia are termed hypogammaglobulinaemia of uncertain significance (HGUS). These patients require long-term follow-up, as some will evolve into CVID.

Amelioration of Murine Passive Immune Thrombocytopenia by IVIg and a Therapeutic Monoclonal CD44 Antibody Does Not Require the Myd88 Signaling Pathway

Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by a low platelet count and the production of anti-platelet antibodies. The majority of ITP patients have antibodies to platelet integrin αIIbβ3 (GPIIbIIIa) which can direct platelet phagocytosis by macrophages. One effective treatment for patients with ITP is intravenous immunoglobulin (IVIg) which rapidly reverses thrombocytopenia. The exact mechanism of IVIg action in human patients is unclear, although in mouse models of passive ITP, IVIg can rapidly increase platelet counts in the absence of adaptive immunity. Another antibody therapeutic that can similarly increase platelet counts independent of adaptive immunity are CD44 antibodies. Toll-like receptors (TLRs) are pattern recognition receptors which play a central role in helping direct the innate immune system. Dendritic cells, which are notable for their expression of TLRs, have been directly implicated in IVIg function as an initiator cell, while CD44 can associate with TLR2 and TLR4. We therefore questioned whether IVIg, or the therapeutic CD44 antibody KM114, mediate their ameliorative effects in a manner dependent upon normal TLR function. Here, we demonstrate that the TLR4 agonist LPS does not inhibit IVIg or KM114 amelioration of antibody-induced thrombocytopenia, and that these therapeutics do not ameliorate LPS-induced thrombocytopenia. IVIg was able to significantly ameliorate murine ITP in C3H/HeJ mice which have defective TLR4. All known murine TLRs except TLR3 utilize the Myd88 adapter protein to drive TLR signaling. Employing Myd88 deficient mice, we found that both IVIg and KM114 ameliorate murine ITP in Myd88 deficient mice to the same extent as normal mice. Thus both IVIg and anti-CD44 antibody can mediate their ameliorative effects in murine passive ITP independent of the Myd88 signaling pathway. These data help shed light on the mechanism of action of IVIg and KM114 in the amelioration of murine ITP.

Inflammatory myopathies

The inflammatory myopathies include polymyositis, dermatomyositis, necrotizing autoimmune myopathy (NAM), and inclusion body myositis (IBM). On the basis of clinical experience, most patients respond to corticosterioids to some degree or for a time period. For patients insufficiently responding or for steroid-sparing, the treatment options vary among practitioners, generating a genuine uncertainty. This timely review highlights emerging new therapies and provides practical therapeutic algorithms. For patients insufficiently responding to corticosteroids, the commonly used immunosuppressants, such as azathioprine, mycophenolate, methotrexate, or cyclosporine, may exert a nonevidence-based 'steroid-sparing' effect but provide minimal benefit on their own. The second line therapy is intravenous immunoglobulin (IVIg) based on a controlled study conducted in dermatomyositis; the drug is also effective in many patients with polymyositis and NAM. Rituximab and tacrolimus may offer additional benefit. Anti-TNF agents are disappointing. IBM remains difficult to treat; although early on some patients may partially respond to steroids or IVIg, they soon become unresponsive and the disease progresses. Emerging agents against T cells, B cells, and transmigration molecules are discussed as promising therapeutic options. New biological agents are in the offing for control trials. Appropriate outcome measures are however needed to assess and monitor responses.

Immunotherapy of Inflammatory Myopathies: Practical Approach and Future Prospects

The inflammatory myopathies, a group of chronic myopathic conditions, are potentially treatable, so proper diagnosis and early initiation of therapy are necessary. The most common types are polymyositis (PM), dermatomyositis (DM), necrotizing autoimmune myopathy (NAM), and inclusion body myositis (IBM). This review provides practical advice on treatment and identifies emerging new therapies. Although IBM is difficult to treat, PM, DM, and NAM respond to appropriate immunotherapies, if diagnosed early and treated aggressively. In uncontrolled studies, PM and DM respond to prednisone to some degree and for a period of time. The commonly used immunosuppressive drugs (azathioprine, cyclosporine, mycophenolate, or methotrexate) may offer some non-evidence-based "steroid-sparing" effect but provide minimal benefit on their own. As a result, the second-line therapy is intravenous immunoglobulin (IVIg), which a controlled study has shown to be effective in DM and which appears to be effective in PM and NAM; it offers minimal and transient benefit to only a small number of IBM patients, however. Uncontrolled series have suggested that rituximab and tacrolimus may offer additional benefit to some patients not adequately controlled with the aforementioned therapies. IBM is usually resistant to most therapies, but early initiation of therapy may be helpful at times. Emerging agents against T cells, B cells, transmigration, or transduction molecules are discussed as potential new treatment options.