Chapter 62 - Dermatomyositis Flashcards by Caryl Lyca Dematawaran

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Diagnosis of cutaneous dermatomyositis requires:
1. Presence of ___ major criteria or ___ major criterion and ___ minor criteria and
2. _____ consistent with cutaneous dermatomyositis

Diagnosis of cutaneous dermatomyositis requires:
1. Presence of two major criteria or one major criterion and two minor criteria and
2. Skin biopsy changes consistent with cutaneous dermatomyositis

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Heliotrope sign

Major Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Gottron papules

Major Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Gottron sign

Major Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Macular violaceous erythema involving various areas

Minor Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Nailfold capillary telangiectasia, hemorrhage-infarct

Minor Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Poikiloderma

Minor Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Mechanic’s hands

Minor Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Cutaneous calcinosis

Minor Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Cutaneous ulcers

Minor Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Pruritus

Minor Criteria

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema
(each area counts as ___ minor criterion)

Macular violaceous erythema
(each area counts as one minor criterion)

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema in the V-area of neck or upper chest

V-neck sign

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema in the posterior neck or posterior shoulders

Shawl sign

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TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema in the lateral thighs or hips

Holster sign

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TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

Most frequent, up to 20%, anti-tRNA synthetase

Jo-1: histidyl

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TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Increased risk of ILD
PL-7: mild skin or muscle
disease
PL-12, KS, OJ: isolated ILD
Variable spectrum of findings of the antisynthetase syndrome: ILD, fever, arthritis, myositis, mechanic’s hands, Raynaud phenomenon

Anti-tRNA synthetase

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TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Hallmark cutaneous disease, good prognosis and response to therapy; relapses common; high CK values (>5000 U/L); rare ILD, cancer; not amyopathic

Anti–Mi-2

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Increased cancer risk in adults
Severe cutaneous disease
Red-on-white skin lesions
Ovoid palatal patch
Low ILD risk
Low CK values (200–2000 U/L)

Anti–transcriptional intermediary factor (TIF1)-γ

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
High ILD risk, RP-ILD, especially in Asians
Skin ulceration, red palmar papules, alopecia, gingival pain
Arthritis
Often isolated high aldolase

Anti–melanoma differentiation–associated gene 5 (MDA5)

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Increased cancer risk in adults
Increased risk of calcinosis
Peripheral edema, myalgia, severe dysphagia
Distal weakness

Anti–nuclear matrix protein 2 (NXP2)

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Skin disease onset before myositis
May have severe skin disease
Dysphagia

Anti–small ubiquitin-like modifier activating enzyme (SAE)

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

PHENOTYPE IN JUVENILE DERMATOMYOSITIS
Older onset
Antisynthetase syndrome with ILD, fever, nonerosive polyarthritis, Raynaud phenomenon, mechanic’s hands

Anti-tRNA synthetase

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

PHENOTYPE IN JUVENILE DERMATOMYOSITIS
Classic skin disease
Myositis
Good therapeutic response; more likely to be in remission at 2 yr

Anti–Mi-2

TABLE 62-2 Clinical Associations with Autoantibodies Associated with Dermatomyositis AUTOANTIBODY PHENOTYPE IN JUVENILE DERMATOMYOSITIS Severe skin disease Lipodystrophy

Anti–transcriptional intermediary factor (TIF1)-γ

TABLE 62-2 Clinical Associations with Autoantibodies Associated with Dermatomyositis AUTOANTIBODY PHENOTYPE IN JUVENILE DERMATOMYOSITIS ILD in Japanese cohort Skin ulceration, arthritis, milder myositis

Anti–melanoma differentiation–associated gene 5 (MDA5)

TABLE 62-2 Clinical Associations with Autoantibodies Associated with Dermatomyositis AUTOANTIBODY PHENOTYPE IN JUVENILE DERMATOMYOSITIS Calcinosis, more severe myositis, poorer functional status

Anti–nuclear matrix protein 2 (NXP2)

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Photoprotection

First line for skin disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Topical steroids

First line for skin disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Hydroxychloroquine or Chloroquine

First line for skin disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Quinacrine

First line for skin disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Systemic corticosteroids

First line for muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Methotrexate

Second line for skin and muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Mycophenolate mofetil

Second line for skin and muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Intravenous immune globulin

Second line for skin and muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Azathioprine

Second line for skin and muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Tofacitnib

Third line for skin disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Leflunomide

Third line for skin and muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Dapsone

Third line for skin disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Thalidomide

Third line for skin disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Cyclosporine

Third line for skin and muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Cyclophosphamide

Third line for skin and muscle disease

TABLE 62-4 Treatment Ladder in Dermatomyositis SKIN DISEASE vs MUSCLE DISEASE First Line vs Second Line vs Third Line Rituximab

Third line for muscle disease