What is the role of IVIG in the treatment of myositis?
Some physicians also use IVIg alone as first-line therapy in patients who have necrotizing myopathy with the anti-HMGCR autoantibody. Some physicians use IVIg to treat inclusion body myositis, although the effectiveness of this is questionable. Nevertheless, some patients who have dysphagia (swallowing problems) do seem to benefit.
Is IVIG a first-line treatment for statin-triggered autoimmune myopathy?
The use of IVIG can be associated with serious adverse effects, including anaphylaxis, thromboembolic events, transfusion-associated lung injury, and others. Thus, IVIG therapy must be used cautiously.5However, our experience suggests that monotherapy with IVIG may be considered as a first-line treatment for statin-triggered autoimmune myopathy.
What is immune-mediated necrotizing myopathy (imnm)?
Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. Here, we will review the characteristics of patients with IMNM. Recent Findings
What should I know before IVIg is started?
Before IVIg is started, the patient should be tested to see if they have IgA in their blood. Up to 5% of the general population has an IgA deficiency, and if you have it and you receive a preparation with IgA antibodies, then a reaction may occur.
How do you treat necrotizing myopathy?
Treatment includes high-dose corticosteroids, early administration of intravenous immunoglobulin (IVIG), plasmapheresis, and immunotherapy with methotrexate, mzathioprine, rituximab, cyclophosphamide, and mycophenolate mofetil, addressing the underlying cause if any.
Does IVIg help myositis?
In conclusion, our findings indicate that IVIg can be effective as third line therapy in the treatment of patients with dermatomyositis, polymyositis, or overlap syndromes whose disease is not adequately controlled by corticosteroids and other immunosuppressive agents but is not usually effective in patients with ...
How does IVIg help dermatomyositis?
Infusion Medications for DM Currently there are two primary infusion therapy options for DM: Intravenous immunoglobulin (IVIg) treatments and Rituximab (Rituxan) infusion. IVIg treatments are derived from human plasma containing antibodies that work to suppress the DM antibodies that harm the muscles and skin.
How is autoimmune myopathy diagnosed?
Muscle biopsy should be considered in the diagnostic workup of all patients with autoimmune myopathy. In patients with characteristic clinical features of DM, a skin biopsy confirming the diagnosis of DM may be sufficient for the diagnosis.
How can I tell if IVIG is working?
When Can I See the Results? IVIG patients often begin to see results from their treatments anywhere from 6 months to a year. During this time, your doctor will closely monitor and track to see if there are improvements in relieving the symptoms associated with your primary diagnosis.
Does IVIG help with muscle weakness?
Intravenous immunoglobulin therapy (IVIg 0.4 g/kg×5 days) markedly reduced the severe pain and muscle weakness in the legs. Eventually, pain assessed by the Visual Analogue Scale was relieved by 80% and muscle strength was also well recovered, thereby enabling the patient to walk with a cane.
How long does it take IVIG to work for dermatomyositis?
Additionally, the study affirms that we can determine whether a patient will have a good response to IVIG in just two to three months — after an average 1.82 cycles of the treatment — which is important given the treatment's cost and its burden on patients.
What is necrotizing myopathy?
Necrotizing myopathy is a newly defined form of idiopathic inflammatory myopathy, or myositis. Patients with necrotizing myopathy have muscle biopsies that show much less inflammation in the muscle tissue than polymyositis patients, but they have increased evidence of muscle cell death, or necrosis.
How quickly does IVIG work?
Each person's response to IVIg varies. If IVIg is to have an effect on your myasthenia, it may take up to 4 weeks for you to notice any improvement in your symptoms. Some people, however, do not respond to this treatment. If this is the case, you and your doctor will discuss alternatives.
Can you recover from necrotizing myopathy?
Even with treatment, recovery for many patients is poor. In his research, Allenbach notes that “the outcome of anti-SRP myopathy is poor since only half of the patients recover normal strength after four years.”
How long can you live with necrotizing myopathy?
For dermatomyositis, polymyositis, and necrotizing myopathy, the progression of the disease is more complicated and harder to predict. More than 95 percent of those with DM, PM, and NM are still alive more than five years after diagnosis.
Is necrotizing autoimmune myopathy fatal?
Necrotising myopathy is a rare but fatal aetiology in patient's presenting with weakness and shortness of breath. Patients can have variable presentations and may initially present with symptoms other than skeletal muscle weakness.
Is IVIG effective for dermatomyositis?
However, it is often especially effective for patients with refractory dermatomyositis skin symptoms and patients with interstitial lung disease.
Can IVIG be used for inclusion body myositis?
Some physicians use IVIg to treat inclusion body myositis, although the effectiveness of this is questionable. Nevertheless, some patients who have dysphagia (swallowing problems) do seem to benefit.
What are the adverse reactions of IVIG?
Minor adverse reactions occurred in five of the 16 patients: local erythema in two, itching in one, erythematous rash in one , and headache in one. More serious complications occurred after the first course of treatment in two patients: a mild cortical stroke resulting in transient aphasia and right upper limb weakness in one (7) and staphylococcal septicaemia in another (5), resulting in cessation of IVIg therapy.
How long is inclusion body myositis?
INCLUSION BODY MYOSITIS. Each of the five patients with inclusion body myositis had longstanding disease of five to 17 years duration and were incapacitated mainly because of quadriceps and hand weakness as well as more widespread limb weakness.
Is IVIG effective for neurological disorders?
High dose intravenous immunoglobulin (IVIg) is effective in the treatment of various immune mediated neurological and other disorders. 3-6 There have been several reports of IVIg therapy in patients with inflammatory myopathies 7-16 but experience with this treatment is still limited. Most reports have been based on uncontrolled studies in which ...
Is IVIG effective for dermatomyositis?
It is relevant that IVIg alone was not found to be effective when used as initial treatment in an open study of 11 patients with polymyositis or dermatomyositis. 14 IVIg may therefore be more effective in patients who have already had or are on concurrent corticosteroid or other immunosuppressive therapy.
Abstract
We explored efficacy and safety of IVIg as first-line treatment in patients with an idiopathic inflammatory myopathy.
Introduction
Glucocorticoids, the first-line treatment in patients with an idiopathic inflammatory myopathy (IIM), have insufficient treatment efficacy.
Methods
From March 2017 to January 2019, we consecutively included patients diagnosed at three referral centres for IIM in The Netherlands (Amsterdam, Rotterdam, Nijmegen).
Results
We consecutively screened 59 patients with newly diagnosed IIM and included 20 patients ( Fig. 2 ). The most common reasons for non-eligibility were the use of prior immunosuppressants at a higher dose than predefined ( n = 18), not meeting the minimal disability criterion ( n = 11) and a disease duration of >9 months ( n = 4).
Discussion
We found that first-line IVIg monotherapy induced at least moderate improvement in nearly half of patients with newly diagnosed IIM. The IMACS has defined different categories of response but there is as of yet no consensus on which level of improvement should be considered as clinically relevant.
Acknowledgements
We thank R. Aggarwal for his help regarding the implementation of the 2016 ACR/EULAR TIS and M.D.J. Wolvers for her help with the statistical analyses.