Table 2

Scope and key recommendations of available CPGs for IIMs

ScopeFirst author, date of publication (ref)Key recommendations of the CPG
General managementSunderkotter, 201631
  • All patients with IIMs should undergo a pulmonary function test and—in case of pathological findings—further pulmonology workup.

  • Tumour screening in DM with anti-TIF1-γ include 18-fluoro-deoxyglucose-positron emission tomography/CT or CT of the thorax and abdomen in combination with a gynaecological/urological examination is recommended (no strategy is provided in other case by the CPG).

  • Treatment of IIMs includes:

    • CS (intravenous if severe, oral if not).

    • AZA (for adult) and MTX (for children) when: (1) severe IIMs, (2) impossibility to reduce CS dosage below the ‘Cushing threshold’ after 3 months.

    • IVIG is recommended in patients unresponsive to CS+AZA.

    • Non-pharmacological measures: regular physical therapy, sun protection (for patients with DM).

Enders, 201720
  • All children with suspected IIMs should be referred to a specialised centre. High-risk patients (as defined in the CPG) need immediate/urgent referral.

  • Assessment of organ involvement (muscle, skin, lung, heart), calcinosis and antibodies (see CPG for details) is recommended for all children with IIMs.

  • Disease activity, damages and health status should be monitored in a standardised way.

  • Juvenile IIMs treatment includes:

    • Sun protection, exercise programme.

    • First line: high-dose CS and MTX.

    • If failure (considered within the first 12 weeks): topical TACRO/ CS (if localised skin disease), CsA or MMF (if intolerance to MTX), IVIG as adjunct or RTX as adjunct or CYC or antitumour necrosis factor therapies (if resistance).

Treatment (overall)Drake, 199619
  • Non-pharmacological treatments of IIMs include physical therapy, photoprotection and adequate nutrition.

  • Pharmacological treatments of IIMs include CS (topical and systemic); antimalarial and CS-sparing agents (no limitative list provided in the CPG).

  • Calcinosis treatments include medical (diphosphonates, aluminium hydroxide, probenecid, colchicine and low-dose warfarin) and surgical management.

Hengstman, 200923
  • First line of IIMs is prednisone (1 mg/kg) and AZA or MTX.

  • Second line, in case of severe pulmonary involvement, is CsA or TACRO or CYC.

  • Second line, in case of no severe pulmonary involvement, is IVIG.

  • Third line is RTX or TACRO or MMF or CsA or CYC.

Treatment (IVIG)Bril, 199916
  • IVIG is favourably recommended for the treatment of DM and recommended as a last resort for the other IIMs.

Feasby, 200721
  • IVIG is recommended as an adjunctive treatment for DM who did not adequately respond to other immunosuppressant medications (such as CS, MTX or AZA).

  • IVIG may be considered as an adjunctive treatment option for PM who failed to respond to first-line therapies.

  • IVIG is not recommended for IBM.

Donofrio, 200917
  • IVIG therapy is recommended as add-on treatment in refractory IIMs.

  • IVIG is not recommended for IBM.

Patwa, 201229
  • IVIG may be considered for the treatment of non-responsive adult DM.

  • Evidence is insufficient to support or refute the use of IVIG in treating IBM and PM.

Enk, 201614Severe forms of DM, PM and IBM are considered by the authors as indication of IVIG, as first-line treatment (in fulminant course, severe myolysis or paralysis) or second-line treatment (in other cases), with continuation of immunosuppressive therapy.
PregnancyDoria, 200418
  • Patients with IIMs should be correctly informed on the risk of becoming pregnant.

  • Pregnancies should be planned when IIMs is in remission

  • Patients with IIMs should be regularly monitored during gestation and postpartum by a multidisciplinary team.

  • In the case of disease relapse, treatment has to be started as soon as possible.

  • IIMs treatment in pregnant patients is: CS (1 mg/kg/day until thenormalisation of serum creatine kinase levels). If insufficient response: CsA, AZA, plasma exchange or IVIG.

Disease measurement (tools)Alexanderson, 200715
  • Disease activity and disability should be measured at disease onset, at 3, 6 and 12 months, and then at least once a year.

  • Only valid and reliable clinical outcome measures should be used (such clinical outcome measures were not available at the time of the publication of the CPG).

Tumour screeningTitulaer, 201132
  • Patients with DM should have CT-thorax/abdomen, ultrasound of the pelvic region and mammography in women, ultrasound of testes in men under 50 years and colonoscopy in men and women over 50.

  • If primary screening is negative, repeat screening after 3–6 months and screen every 6 months up till 4 years.

Extramuscular involvement (skin)Fujimoto, 201622
  • For calcinosis in IIMs: low-dose warfarin, aluminium hydroxide gel, diltiazem hydrochloride, probenecid or bisphosphonate are recommended as an option. Surgical treatment is also recommended as an option.

  • For panniculitis in IIMs: CS is recommended. If no response, immunosuppressants such as CsA, MTX and AZA are recommended as an option.

Extramuscular involvement (lung)Morrisset, 201628*
  • In acute or severe IIMs–ILD: high-dose steroids+CYC or RTX or CsA or TACRO is recommended.

  • In chronic or mild to moderate IIMs–ILD: steroids+MMF or AZA is recommended.

  • In case of failure: it is recommended to switch agent or consider combination of agents, or consider IVIG or consider transplantation referral.

  • AZA, azathioprine; CPGs, clinical practice guidelines; CS, corticosteriod; CYC, cyclophosphamide; CsA, ciclosporin A; DM, dermatomyositis; IBM, inclusion body myositis; IIM: idiopathic inflammatory myopathy; ILD: interstitial lung disease; IVIG, intravenous immunoglobulin; MMF, mycophenolate mofetil; MTX, methotrexate; PM, polymyositis; RTX, rituximab; TACRO, tacrolimus.