I think excess water intake
Cut on sugar
Cut on fat
Moderate intake of salt and
Cleanliness
Steroids are first line therapy. steroid sparing agents like azathioprine/methotrexate (if no ILD)/Mycophenolate should be added to minimise steroids to lowest possible doses.i find good effect of Rituximab in resistant and/or aggressive cases.
Regards
I'm totally agree with Bijit, but reading Rituximab, which is the best effective doses 500mg or 1000mg then after 2 weeks or 6 months we can repeat the same dose?
500 mg X4 or 1g X 2 would be equally ok. i give 1g X 2. after 6 months, i repeat the dose. The third course I give only when the patient becomes symptomatic.
When dermatomyositis is severe, two considerations are important:
A. To rule out concomitant malignancy (Ovarian malignancy is the most common, followed by lung, GI tract and breast cancers, and non-Hodgkin’s lymphoma). In 58% of cases, the malignancy was discovered after the diagnosis of DM was made, usually within the first year. Dermatomyositis will not respond to treatment if cancer is present.
B.Treatment must be aggressive.
1.Pulsed intravenous methylprednisolone 0.5–1 g daily for 3 Days monthly
2. Pulsed intravenous Cyslophosphamide 500-750 mg/m2 monthly . First pulse given on day 4 after third day of bolus methylprednisolone
Once remission is achieved, maintenance with azathioprine 2–3 mg/kg/day can be given with close monitoring for relapse.
More recent treatment available is Rituximab.
Dear Dr Majda,
CyP is not favoured in Dermatomyositis as it itself is myotoxic. In our centre we avoid it.
Regards
For Resistant cases IVIG and Rituximab are the best options. As mentioned above you need to rule out Malignancy in these cases .
First line of treating severe DM are 3-5-day "pulses" of steroids to be continued by oral doses of 2mg/kg daily with slowly tappering the dose, afterwards Methotrexate 10-20 mg/m2 weekly or azathioprine 1-3 mg/kg daily or cyclophosphamyde in monthly "pulses" 750 mg-1000 mg, sometimes together with hydroxychlorochin 250-500 mg daily. If not efficient, rituximab should be introduced 500-1000 mg weekly x2 or even x4 if necessary.
Best regards
Lana Tambić Bukovac
A high dose of prednisone or its equivalent (0.75–1 mg/kg/day) is recommended as starting dose, which should be maintained for 4–12 weeks.Dose tapering usually begins after 1 month and is guided by improvements in muscle function and CK reduction. Introduce immunosuppressive agents early in order to facilitate corticosteroids dose tapering.Methotrexate is the most used immunosuppressive drug . It is used in similar dosages as in rheumatoid arthritis, up to 25 mg weekly. Hydroxychloroquine can be added to the treatment
She should avoid exposure to sunlight . The use of sunscreens is mandatory. Application of topical corticosteroids or the more recent class of calcineurin inhibitors, such as tacrolimus may be useful in controlling cutaneous manifestations. . Active physical exercise should be introduced early.
I agree with all said above.
Steroids are the cornerstone of initial therapy to induce remission - IV pule of methyl prednisolone followed by slowly tapering dose of oral Prednisolone.
The choice of the 2nd agent for me is governed by the severity.
Mild cases (no dysphagia, dysphonia, resp involvemnt or calcinosis) - MTX would be the first choice. Azathioprine is an alternative.
Moderate to severe cases - Cyclophosphamide or Rituximab. I tend to prefer Cyclo because if Ritux does not work it still stay in the system for a long time & if one adds on Cyclo, that will effectively knock out both B & T cells. Once we induce remission with Cyclo, I will try to maintain it with MTX or Aza.
If it is just skin that is severe with calcinosis, pre calcinotic nodules - IVIG or Infliximab are options; Hydroxychloroquin is a useful adjunct in these situations.
I personally don't have much experience with MMF or Ciclosporin.
Supportive management would include factor 50 sunscreen, physio etc.
Thanks
Kishore.
pulse steroids, IVIG and Rituximab. avoid cyclo because of reproductive issues. mtx and imuran as steroid sparing
prednisolone, intervenous immunoglobins, plasmapheresis and mld dose of chemotherapy ( Methotrexate and HCQS) Mycophenolate( for steroid sparing effect, AzothioprimeCyclophosphamide ( as Chemotherapy ) I have little experience with Infliximab/Rituximab as they are costly and in a country where TB is a pandemic disease
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