Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

severe disease or acute flares

1st line – intravenous corticosteroids

Back
ACUTE
|
severe disease or acute flares
1st line – 

intravenous corticosteroids

Severe muscle disease: includes those with severe muscle weakness (e.g., quadriplegia), interstitial lung disease, myocarditis, respiratory failure, severe dysphagia, or other life-threatening complications.

In severe skin disease or acute flares, short-term use of systemic corticosteroids may be indicated until other treatments take effect. Patients are subsequently transferred to high-dose oral corticosteroids. To minimize toxicity, it is recommended to always use the lowest possible dose and taper dose once response is achieved.[72]

Primary options

methylprednisolone sodium succinate: 1 g intravenously once daily for 3-5 days

Consider – intravenous immunoglobulin (IVIG)

Back
ACUTE
|
severe disease or acute flares
Consider – 

intravenous immunoglobulin (IVIG)

Treatment recommended for SOME patients in selected patient group

IVIG combined with an intravenous corticosteroid is sometimes used initially rather than a corticosteroid alone, depending on the physician's experience.[125][126]

If anti-signal recognition particle (SRP) antibodies are present in patients with DM, they are associated with acute severe, treatment-resistant necrotizing myositis.[46] IVIG is often required at an early disease stage in these patients.[93]

Guidance from the Department of Health in the UK recommends adjunct IVIG for patients with DM who have significant muscle weakness.[145][146]​​

Small studies and case reports have demonstrated the benefit of adjunctive IVIG in both resistant skin and muscle disease.[147][148][149][150]

Although it is well tolerated, there have been reports of acute renal failure, skin rashes, aseptic meningitis, and stroke associated with treatment.

Primary options

immune globulin (human): 2 g/kg intravenously per month given in divided doses over 5 days

Consider – immunosuppressant

Back
ACUTE
|
severe disease or acute flares
Consider – 

immunosuppressant

Treatment recommended for SOME patients in selected patient group

Patients diagnosed with antisynthetase syndrome may be corticosteroid-resistant and concomitant use of immunosuppressive drugs is required.[45]

If anti-signal recognition particle (SRP) antibodies are present in patients with DM, they are associated with acute severe, treatment-resistant necrotizing myositis.[46] Immunosuppressive drugs are often required at an early disease stage.[93]

The presence of anti-melanoma differentiation-associated gene 5 (MDA5) is specific to clinically amyopathic DM, and is strongly associated with rapidly progressive interstitial lung disease. Patients often require treatment with immunosuppressive drugs as well as high-dose systemic corticosteroids from an early disease stage.[47]

Primary options

methotrexate: 7.5 mg orally once weekly on the same day each week initially, increase by 2.5 mg/week increments at monthly intervals according to response, maximum 20 mg/week

OR

azathioprine: 2 mg/kg/day orally

OR

mycophenolate mofetil: 1 g orally twice daily

OR

cyclosporine modified: 2.5 to 5 mg/kg/day orally

OR

cyclophosphamide: 1-3 mg/kg/day orally

OR

tacrolimus: consult specialist for guidance on dose

Consider – rituximab

Back
ACUTE
|
severe disease or acute flares
Consider – 

rituximab

Treatment recommended for SOME patients in selected patient group

If anti-signal recognition particle (SRP) antibodies are present in patients with DM, they are associated with acute severe, treatment-resistant necrotizing myositis.[46] Rituximab may often be required at an early disease stage.[93]

Primary options

rituximab: consult specialist for guidance on dose

ONGOING

combined muscle and skin disease

1st line – oral corticosteroid

Back
ONGOING
|
combined muscle and skin disease
1st line – 

oral corticosteroid

In patients with myositis, high doses of oral corticosteroids are recommended as initial treatment. They reduce morbidity and improve muscle strength and motor function.[127][130]

To minimize toxicity it is recommended to always use the lowest possible dose and taper dose once response is achieved.[72]

Skin disease often responds to short-term use of corticosteroids but flares when corticosteroid dose is tapered. Other treatments should be added to control skin disease in this setting rather than increasing corticosteroid dose.

Primary options

prednisone: 0.75 to 1.5 mg/kg/day orally for 2-4 weeks, then taper gradually

Plus – photoprotection

Back
ONGOING
|
combined muscle and skin disease
Plus – 

photoprotection

Treatment recommended for ALL patients in selected patient group

Not all patients report photosensitivity, but there is evidence that the skin lesions are exacerbated by sun exposure. All patients should use year-round topical high-protection sunscreens providing protection against both ultraviolet A (3+ star rating) and ultraviolet B (sun protection factor 50 or above).

Consider – treatment of skin disease

Back
ONGOING
|
combined muscle and skin disease
Consider – 

treatment of skin disease

Treatment recommended for SOME patients in selected patient group

In cases where the skin manifestations are unresponsive to therapy for myositis, alternative treatments should be added rather than increasing the corticosteroid dose.

Topical corticosteroids are indicated in patients with cutaneous disease and are usually beneficial even in patients who are already being treated with systemic corticosteroids for myositis. They help control erythema and pruritus, and can provide adequate relief when used alone in some patients.[155][156] Topical corticosteroids of different potencies may be used in combination depending on the patient's symptoms. Potent corticosteroids (e.g., betamethasone valerate 0.1%) and very potent corticosteroids (e.g., clobetasol propionate 0.05%) are often used to treat the trunk and limbs including the hands, as well as the scalp. Moderate potency corticosteroids (e.g., triamcinolone acetonide 0.1%) are used in areas more prone to atrophy such as the face and neck. Mild potency corticosteroids (e.g., hydrocortisone 1%) are typically reserved for the eyelids, although may prove insufficient. Scalp involvement may be treated with foam or lotion formulations.[72][156]

Adjunct treatment with topical antipruritics, simple moisturizers, emollients (e.g., white soft paraffin 50:50 ointment in emollient), and topical formulas containing menthol, phenol, or camphor may provide some relief from pruritus, as well as topical antihistamines such as doxepin.[156]

A trial of an oral antihistamine is indicated for patients with severe pruritus unresponsive to topical treatments. Pruritus is often a nocturnal symptom; therefore, long-acting sedating antihistamines (e.g., hydroxyzine) are useful. Nonsedating antihistamines (e.g., cetirizine) are preferred for daytime use.[156]

Topical tacrolimus is indicated as an adjunct when there is an inadequate response to first-line treatments. There are anecdotal reports in the literature of improvement in pruritus, eczematous components, and poikiloderma.[161][162] However, randomized trials are required. Minimal systemic absorption of drug gives a favorable safety profile.[163]

For patients with skin manifestations unresponsive to topical treatments, oral antimalarials can be used. Hydroxychloroquine has been shown to improve skin lesions and scalp pruritus with no effect on myositis.[157][158][159] Chloroquine is also of benefit, although the risk of retinopathy is greater than with hydroxychloroquine, and therefore hydroxychloroquine is the preferred antimalarial.[160]

Methotrexate is indicated in patients with ongoing disease despite topical treatments and antimalarials, and may be used in combination with these treatments. Methotrexate has been shown to be effective for both muscle and skin disease in DM.[129][130][132][133][164]

For patients with cutaneous disease resistant to methotrexate, there are several case reports of response to other immunosuppressant treatments.[135][138][139][166][169][170][171][172] Reports of the efficacy of azathioprine mainly relate to muscle disease but variable improvement in cutaneous disease has been reported.[134][166] Mycophenolate has been shown to be beneficial in patients with recalcitrant skin disease, and cyclosporine in patients with recalcitrant skin disease and early interstitial lung disease.[135][138][139][168][169][170][171][172]

There is anecdotal evidence to support the use of thalidomide or and dapsone in resistant cutaneous DM.[72][173][174]

Primary options

hydrocortisone topical: (1%) apply to affected area(s) once or twice daily

More

OR

triamcinolone topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

clobetasol topical: (0.05%) apply to the affected area(s) twice daily

More

OR

doxepin topical: (5%) apply to the affected area(s) four times daily when required for up to 8 days

Secondary options

hydroxyzine: 25 mg orally three to four times daily when required

OR

cetirizine: 5-10 mg orally once daily when required

OR

tacrolimus topical: (0.1%) apply to the affected area(s) twice daily

OR

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

OR

methotrexate: 7.5 mg orally once weekly on the same day each week initially, increase by 2.5 mg/week increments at monthly intervals according to response, maximum 20 mg/week

OR

azathioprine: 2 mg/kg/day orally

OR

mycophenolate mofetil: 1 g orally twice daily

OR

cyclosporine modified: 2.5 to 5 mg/kg/day orally

Tertiary options

thalidomide: 100-400 mg orally once daily

OR

dapsone: 50-100 mg orally once daily

Consider – treatment of malignancy

Back
ONGOING
|
combined muscle and skin disease
Consider – 

treatment of malignancy

Treatment recommended for SOME patients in selected patient group

When malignancy is present, treatment of underlying malignancy is essential if the disorder is to be controlled. Improvement or complete resolution of DM has been reported in some cases after successful treatment of underlying malignancy.[178]

2nd line – methotrexate or azathioprine

Back
ONGOING
|
combined muscle and skin disease
2nd line – 

methotrexate or azathioprine

Methotrexate or azathioprine may be considered second line in patients who have not already been started on one of these treatments for cutaneous disease.

Methotrexate has been found to be an effective corticosteroid-sparing agent and may be also be effective in myositis refractory to corticosteroids.[129][130][131] Low-dose oral methotrexate is useful for both muscle and cutaneous involvement.[129][130][132][133] It is often preferred over azathioprine because it is considered to have a more rapid benefit.[127]

Azathioprine is the next choice of therapy. Reports of the efficacy of azathioprine mainly relate to muscle disease but variable improvement in cutaneous disease has been reported.[166] It can be as effective and well tolerated as methotrexate, but seems to take longer to take effect (≥6 months).[134]

Each treatment may be used alone or combined with corticosteroids. Patients who do not respond or show minimal response to corticosteroid therapy can continue to take corticosteroids while these drugs are added.

Primary options

methotrexate: 7.5 mg orally once weekly on the same day each week initially, increase by 2.5 mg/week increments at monthly intervals according to response, maximum 20 mg/week

Secondary options

azathioprine: 2 mg/kg/day orally

Plus – photoprotection

Back
ONGOING
|
combined muscle and skin disease
Plus – 

photoprotection

Treatment recommended for ALL patients in selected patient group

Not all patients report photosensitivity, but there is evidence that the skin lesions are exacerbated by sun exposure. All patients should use year-round topical high-protection sunscreens providing protection against both ultraviolet A (3+ star rating) and ultraviolet B (sun protection factor 50 or above).

Consider – treatment of skin disease

Back
ONGOING
|
combined muscle and skin disease
Consider – 

treatment of skin disease

Treatment recommended for SOME patients in selected patient group

In cases where the skin manifestations are unresponsive to therapy for myositis, alternative treatments should be added rather than increasing the corticosteroid dose.

Topical corticosteroids are indicated in patients with cutaneous disease and are usually beneficial even in patients who are already being treated with systemic corticosteroids for myositis. They help control erythema and pruritus, and can provide adequate relief when used alone in some patients.[155][156] Topical corticosteroids of different potencies may be used in combination depending on the patient's symptoms. Potent corticosteroids (e.g., betamethasone valerate 0.1%) and very potent corticosteroids (e.g., clobetasol propionate 0.05%) are often used to treat the trunk and limbs including the hands, as well as the scalp. Moderate potency corticosteroids (e.g., triamcinolone acetonide 0.1%) are used in areas more prone to atrophy such as the face and neck. Mild potency corticosteroids (e.g., hydrocortisone 1%) are typically reserved for the eyelids, although may prove insufficient. Scalp involvement may be treated with foam or lotion formulations.[72][156]

Adjunct treatment with topical antipruritics, simple moisturizers, emollients (e.g., white soft paraffin 50:50 ointment in emollient), and topical formulas containing menthol, phenol, or camphor may provide some relief from pruritus, as well as topical antihistamines such as doxepin.[156]

A trial of an oral antihistamine is indicated for patients with severe pruritus unresponsive to topical treatments. Pruritus is often a nocturnal symptom; therefore, long-acting sedating antihistamines (e.g., hydroxyzine) are useful. Nonsedating antihistamines (e.g., cetirizine) are preferred for daytime use.[156]

Topical tacrolimus is indicated as an adjunct when there is an inadequate response to first-line treatments. There are anecdotal reports in the literature of improvement in pruritus, eczematous components, and poikiloderma.[161][162] However, randomized trials are required. Minimal systemic absorption of drug gives a favorable safety profile.[163]

For patients with skin manifestations unresponsive to topical treatments, oral antimalarials can be used. Hydroxychloroquine has been shown to improve skin lesions and scalp pruritus with no effect on myositis.[157][158][159] Chloroquine is also of benefit, although the risk of retinopathy is greater than with hydroxychloroquine, and therefore hydroxychloroquine is the preferred antimalarial.[160]

There is anecdotal evidence to support the use of thalidomide or and dapsone in resistant cutaneous DM.[72][173][174]

Primary options

hydrocortisone topical: (1%) apply to affected area(s) once or twice daily

More

OR

triamcinolone topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

clobetasol topical: (0.05%) apply to the affected area(s) twice daily

More

OR

doxepin topical: (5%) apply to the affected area(s) four times daily when required for up to 8 days

Secondary options

hydroxyzine: 25 mg orally three to four times daily when required

OR

cetirizine: 5-10 mg orally once daily when required

OR

tacrolimus topical: (0.1%) apply to the affected area(s) twice daily

OR

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

Tertiary options

thalidomide: 100-400 mg orally once daily

OR

dapsone: 50-100 mg orally once daily

Consider – treatment of malignancy

Back
ONGOING
|
combined muscle and skin disease
Consider – 

treatment of malignancy

Treatment recommended for SOME patients in selected patient group

When malignancy is present, treatment of underlying malignancy is essential if the disorder is to be controlled. Improvement or complete resolution of DM has been reported in some cases after successful treatment of underlying malignancy.[178]

3rd line – other immunosuppressants

Back
ONGOING
|
combined muscle and skin disease
3rd line – 

other immunosuppressants

Other immunosuppressants may be considered third line in patients who have not already been started on one of these treatments for cutaneous disease. Indicated in cases of myositis refractory to corticosteroids and second-line agents.

Each treatment may be used alone, combined with corticosteroids, or combined with other immunosuppressants. Patients not responding or showing minimal response to corticosteroid therapy can continue to take corticosteroids while these drugs are added.

Primary options

cyclosporine modified: 2.5 to 5 mg/kg/day orally

OR

mycophenolate mofetil: 1 g orally twice daily

OR

cyclophosphamide: 1-3 mg/kg/day orally

OR

tacrolimus: consult specialist for guidance on dose

OR

chlorambucil: consult specialist for guidance on dose

Plus – photoprotection

Back
ONGOING
|
combined muscle and skin disease
Plus – 

photoprotection

Treatment recommended for ALL patients in selected patient group

Not all patients report photosensitivity, but there is evidence that the skin lesions are exacerbated by sun exposure. All patients should use year-round topical high-protection sunscreens providing protection against both ultraviolet A (3+ star rating) and ultraviolet B (sun protection factor 50 or above).

Consider – intravenous immunoglobulin (IVIG)

Back
ONGOING
|
combined muscle and skin disease
Consider – 

intravenous immunoglobulin (IVIG)

Treatment recommended for SOME patients in selected patient group

Several small studies and case reports have demonstrated the benefit of adjunctive IVIG in both resistant skin and muscle disease.[147][148][149][150]

Although IVIG is well tolerated, there have been reports of acute renal failure, skin rashes, aseptic meningitis, and stroke associated with treatment.

Primary options

immune globulin (human): 2 g/kg intravenously per month given in divided doses over 5 days

Consider – treatment of skin disease

Back
ONGOING
|
combined muscle and skin disease
Consider – 

treatment of skin disease

Treatment recommended for SOME patients in selected patient group

In cases where the skin manifestations are unresponsive to therapy for myositis, alternative treatments should be added rather than increasing the corticosteroid dose.

Topical corticosteroids are indicated in patients with cutaneous disease and are usually beneficial even in patients who are already being treated with systemic corticosteroids for myositis. They help control erythema and pruritus, and can provide adequate relief when used alone in some patients.[155][156] Topical corticosteroids of different potencies may be used in combination depending on the patient's symptoms. Potent corticosteroids (e.g., betamethasone valerate 0.1%) and very potent corticosteroids (e.g., clobetasol propionate 0.05%) are often used to treat the trunk and limbs including the hands, as well as the scalp. Moderate potency corticosteroids (e.g., triamcinolone acetonide 0.1%) are used in areas more prone to atrophy such as the face and neck. Mild potency corticosteroids (e.g., hydrocortisone 1%) are typically reserved for the eyelids, although may prove insufficient. Scalp involvement may be treated with foam or lotion formulations.[72][156]

Adjunct treatment with topical antipruritics, simple moisturizers, emollients (e.g., white soft paraffin 50:50 ointment in emollient), and topical formulas containing menthol, phenol, or camphor may provide some relief from pruritus, as well as topical antihistamines such as doxepin.[156]

A trial of an oral antihistamine is indicated for patients with severe pruritus unresponsive to topical treatments. Pruritus is often a nocturnal symptom; therefore, long-acting sedating antihistamines (e.g., hydroxyzine) are useful. Nonsedating antihistamines (e.g., cetirizine) are preferred for daytime use.[156]

Topical tacrolimus is indicated as an adjunct when there is an inadequate response to first-line treatments. There are anecdotal reports in the literature of improvement in pruritus, eczematous components, and poikiloderma.[161][162] However, randomized trials are required. Minimal systemic absorption of drug gives a favorable safety profile.[163]

For patients with skin manifestations unresponsive to topical treatments, oral antimalarials can be used. Hydroxychloroquine has been shown to improve skin lesions and scalp pruritus with no effect on myositis.[157][158][159] Chloroquine is also of benefit, although the risk of retinopathy is greater than with hydroxychloroquine, and therefore hydroxychloroquine is the preferred antimalarial.[160]

There is anecdotal evidence to support the use of thalidomide or and dapsone in resistant cutaneous DM.[72][173][174]

Primary options

hydrocortisone topical: (1%) apply to affected area(s) once or twice daily

More

OR

triamcinolone topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

clobetasol topical: (0.05%) apply to the affected area(s) twice daily

More

OR

doxepin topical: (5%) apply to the affected area(s) four times daily when required for up to 8 days

Secondary options

hydroxyzine: 25 mg orally three to four times daily when required

OR

cetirizine: 5-10 mg orally once daily when required

OR

tacrolimus topical: (0.1%) apply to the affected area(s) twice daily

OR

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

Tertiary options

thalidomide: 100-400 mg orally once daily

OR

dapsone: 50-100 mg orally once daily

Consider – treatment of malignancy

Back
ONGOING
|
combined muscle and skin disease
Consider – 

treatment of malignancy

Treatment recommended for SOME patients in selected patient group

When malignancy is present, treatment of underlying malignancy is essential if the disorder is to be controlled. Improvement or complete resolution of DM has been reported in some cases after successful treatment of underlying malignancy.[178]

skin disease only

1st line – topical corticosteroid

Back
ONGOING
|
skin disease only
1st line – 

topical corticosteroid

Topical corticosteroids are indicated in patients with cutaneous disease and are usually beneficial even in patients who are already being treated with systemic corticosteroids for myositis. They help control erythema and pruritus, and can provide adequate relief when used alone in some patients.[155][156]

Topical corticosteroids of different potencies may be used in combination depending on the patient's symptoms. Potent corticosteroids (e.g., betamethasone valerate 0.1%) and very potent corticosteroids (e.g., clobetasol propionate 0.05%) are often used to treat the trunk and limbs including the hands, as well as the scalp. Moderate potency corticosteroids (e.g., triamcinolone acetonide 0.1%) are used in areas more prone to atrophy such as the face and neck. Mild potency corticosteroids (e.g., hydrocortisone 1%) are typically reserved for the eyelids, although may prove insufficient. Scalp involvement may be treated with foam or lotion formulations.[72][156]

Adrenal suppression may occur with prolonged use of high-potency topical corticosteroids.

Primary options

hydrocortisone topical: (1%) apply to affected area(s) once or twice daily

More

OR

triamcinolone topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

betamethasone valerate topical: (0.1%) apply to the affected area(s) once or twice daily

More

OR

clobetasol topical: (0.05%) apply to the affected area(s) twice daily

More

Plus – antipruritic

Back
ONGOING
|
skin disease only
Plus – 

antipruritic

Treatment recommended for ALL patients in selected patient group

Topical antipruritics include simple moisturizers, emollients (e.g., white soft paraffin 50:50 ointment in emollient), and topical formulas containing menthol, phenol, or camphor, as well as topical antihistamines such as doxepin.[156]

A trial of an oral antihistamine is indicated for patients with severe pruritus unresponsive to topical treatments. Pruritus is often a nocturnal symptom; therefore, long-acting sedating antihistamines (e.g., hydroxyzine) are useful. Nonsedating antihistamines (e.g., cetirizine) are preferred for daytime use.[156]

Primary options

doxepin topical: (5%) apply to the affected area(s) four times daily when required for up to 8 days

Secondary options

hydroxyzine: 25 mg orally three to four times daily when required

OR

cetirizine: 5-10 mg orally once daily when required

Plus – photoprotection

Back
ONGOING
|
skin disease only
Plus – 

photoprotection

Treatment recommended for ALL patients in selected patient group

Not all patients report photosensitivity, but there is evidence that the skin lesions are exacerbated by sun exposure. All patients should use year-round topical high-protection sunscreens providing protection against both ultraviolet A (3+ star rating) and ultraviolet B (sun protection factor 50 or above).

2nd line – antimalarial

Back
ONGOING
|
skin disease only
2nd line – 

antimalarial

For patients with skin manifestations unresponsive to topical treatments, oral antimalarials can be used.

Hydroxychloroquine has been shown to improve skin lesions and scalp pruritus with no effect on myositis.[157][158][159] Chloroquine is also of benefit, although the risk of retinopathy is greater than with hydroxychloroquine, and therefore hydroxychloroquine is the preferred antimalarial.[160]

Primary options

hydroxychloroquine sulfate: 200-400 mg/day orally given in 1-2 divided doses

Plus – topical tacrolimus

Back
ONGOING
|
skin disease only
Plus – 

topical tacrolimus

Treatment recommended for ALL patients in selected patient group

There are anecdotal reports in the literature of improvement in pruritus, eczematous components, and poikiloderma with topical tacrolimus.[161][162] However, randomized trials are required. Minimal systemic absorption of drug gives a favorable safety profile.[163]

Primary options

tacrolimus topical: (0.1%) apply to the affected area(s) twice daily

Plus – photoprotection

Back
ONGOING
|
skin disease only
Plus – 

photoprotection

Treatment recommended for ALL patients in selected patient group

Not all patients report photosensitivity, but there is evidence that the skin lesions are exacerbated by sun exposure. All patients should use year-round topical high-protection sunscreens providing protection against both ultraviolet A (3+ star rating) and ultraviolet B (sun protection factor 50 or above).

3rd line – immunosuppressants

Back
ONGOING
|
skin disease only
3rd line – 

immunosuppressants

Indicated in patients unresponsive to topical treatments and antimalarials.

Methotrexate has been shown to be effective for both muscle and skin disease in DM.[129][130][132][133][164] Onset of action is slow and it may be 12 weeks before response is seen. Individual dose titration is required to achieve maximal response. Oral ulceration and nausea may occur and should be treated symptomatically or by dose reduction. Marrow and hepatic toxicity are potentially serious adverse effects and regular monitoring is indicated.[165] Excess alcohol consumption should be avoided. Methotrexate pneumonitis is a potentially fatal complication that may be difficult to detect in patients with interstitial lung disease.

For patients with cutaneous disease resistant to methotrexate, there are several case reports of response to other immunosuppressant treatments.[135][138][139][166][169][170][171][172]

Mycophenolate appears to be well tolerated.[168]

Reports of the efficacy of azathioprine mainly relate to muscle disease but variable improvement in cutaneous disease has been reported.[166] Tolerability of azathioprine may limit its usefulness, as drug withdrawal is common.[167]

Cyclosporine may have particular benefit in the subset of patients with interstitial lung disease but only in the early inflammatory stage of disease.[172] Patients should be counseled regarding increased susceptibility to infection.

Primary options

methotrexate: 7.5 mg orally once weekly on the same day each week initially, increase by 2.5 mg/week increments at monthly intervals according to response, maximum 20 mg/week

Secondary options

mycophenolate mofetil: 1 g orally twice daily

Tertiary options

azathioprine: 2 mg/kg/day orally

OR

cyclosporine modified: 2.5 to 5 mg/kg/day orally

Plus – photoprotection

Back
ONGOING
|
skin disease only
Plus – 

photoprotection

Treatment recommended for ALL patients in selected patient group

Not all patients report photosensitivity, but there is evidence that the skin lesions are exacerbated by sun exposure. All patients should use year-round topical high-protection sunscreens providing protection against both ultraviolet A (3+ star rating) and ultraviolet B (sun protection factor 50 or above).

3rd line – thalidomide, dapsone, or intravenous immunoglobulin (IVIG)

Back
ONGOING
|
skin disease only
3rd line – 

thalidomide, dapsone, or intravenous immunoglobulin (IVIG)

There is anecdotal evidence to support the use of thalidomide and dapsone in resistant cutaneous DM.[72][173][174] The mechanisms by which these drugs exert their effects are not fully understood.

Teratogenicity is the major adverse effect associated with the use of thalidomide, and patient counseling and education regarding the avoidance of pregnancy is mandatory.[175] Peripheral neuropathy is a potential serious adverse effect of treatment with thalidomide and should be actively sought with serial neurophysiologic assessment.[176]

Dapsone is generally well tolerated at low dose but hematologic toxicity, gastrointestinal disturbance, and urticaria may limit its use. Co-prescription of cimetidine may reduce the risk of hematologic complications.[179]

Guidance from the Department of Health in the UK recommends adjunct IVIG for patients with DM with refractory skin involvement.[145][146]​​ Small studies and case reports have demonstrated the benefit of adjunctive IVIG in both resistant skin and muscle disease.[147][148][149][150]

Although IVIG is well tolerated, there have been reports of acute renal failure, skin rashes, aseptic meningitis, and stroke associated with treatment.

Primary options

thalidomide: 100-400 mg orally once daily

OR

dapsone: 50-100 mg orally once daily

OR

immune globulin (human): 2 g/kg intravenously per month given in divided doses over 5 days

Plus – photoprotection

Back
ONGOING
|
skin disease only
Plus – 

photoprotection

Treatment recommended for ALL patients in selected patient group

Not all patients report photosensitivity, but there is evidence that the skin lesions are exacerbated by sun exposure. All patients should use year-round topical high-protection sunscreens providing protection against both ultraviolet A (3+ star rating) and ultraviolet B (sun protection factor 50 or above).

Consider – treatment of malignancy

Back
ONGOING
|
skin disease only
Consider – 

treatment of malignancy

Treatment recommended for SOME patients in selected patient group

When malignancy is present, treatment of underlying malignancy is essential if the disorder is to be controlled. Improvement or complete resolution of DM has been reported in some cases after successful treatment of underlying malignancy.[178]

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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