62 - Dermatomyositis Flashcards by dvojnd rivera

Have the characteristic rash of dermatomyositis but no clinical signs or symptoms of myositis

Clinically amyopathic dermatomyositis

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Have the characteristic DM rash but no clinical signs or symptoms of myositis

Clinically amyopathic dermatomyositis (CADM)

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_____% of patients have CADM

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If not CADM, then the rest of patients with DM have

Classic dermatomyositis

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CADM can be further subclassified into

Hypomyopathic CM

Amyopathic DM

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Hypomyopathic DM is classified if one of these test results is abnormal

Magnetic resonance imaging
Electromyography
Muscle biopsy
Laboratory studies of muscle enzymes

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CADM in which all the test results are normal

Amyopathic DM

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Y/N: It has become clear that muscle histology and clinical findings can be defined that are specific to DM

No - cannot be defined

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Three major criteria in Sontheimer’s proposed diagnostic criteria for cutaneous dermatomyositis

Heliotrope sign
Gottron papules
Gottron sign

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Violaceous erythema over the eyelids

Heliotrope sign

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Papules overlying the MCP and IP joints

Gottron papules

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Erythema overlying the knees, elbows, IP joints, olecranon processes, patellae, and medial malleoli

Gottron sign

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Sontheimer suggested that a diagnosis of CADM could be made if _____ major criteria were present or _____ major and _____ minor criteria were present in addition to biopsy of at least one region showing histopathologic changes consistent with DM

1, 2

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CADM is an important group to recognize because these patients appear to harbor the same risks for _____ as their classic counterparts

Systemic disease

Malignancy

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DM has a bimodal distribution in the age of disease onset, occurring at two peaks, at _____ years and _____ years of life

5 to 14

45 to 64

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DM triggers

Substantial ultraviolet exposure
Strenuous activity (for the patient with concurrent myositis)
Recent malignancy

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There is often significant pruritus associated with affected skin, particularly on the _____, which may also be described as a “tightness” or burning or with other dysesthetic qualities such as crawling or tingling

Scalp

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Characteristic cutaneous feature of affected skin in DM

Violaceous patches and plaques, varying from a bright pink to a deep violet color

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Trunk involvement seen on the posterior neck, upper back, and shoulders, which may extend to the posterior upper arms

Shawl sign

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Confluent violaceous erythema on the sun-exposed areas of the lower anterior neck and anterior chest

V-neck sign

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Generally, (juvenile/adult) DM more characteristically displays atrophy and poikiloderma in classic areas of Gottron sign

Juvenile

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Violaceous erythema ad poikiloderma on the lateral hips and lateral thighs

Holster sign

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Hyperkeratosis and fissuring along the medial thumb and lateral second and third digits

Mechanic’s hands

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Mechanic’s hands is a cutaneous clue to the possible presence of

Interstitial lung disease

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Proximal nailfold involvement can be highly suggestive of

Ulceration may be present in 30% of patients and often affects the skin over the _____ although it can be found anywhere

Extensor joint surfaces

Cutaneous ulceration in DM warrants concern for the presence of

Anti-MDA5 antibodies or | Malignancy

Ulcers have been correlated with the presence of _____, but this is likely through their association with anti-MDA5 antibodies

Interstitial lung disease

Symmetric violaceous patch across the midline of the hard palate

Ovoid palatal patch

Ovoid palatal patch is most frequently observed in the subset of patients with DM with

Anti-transcriptional intermediary factor 1gamma (TIF1-gamma)

Ovoid palatal patch could be confused with oral findings of discoid lupus or lichen planus, but their consistent localization to the _____ may aid in the diagnosis of DM

Center of the hard palate

Although _____ is often an important sign of activity, it can often be a sign of damage as well

Erythema

Distinctive and pathognomonic pattern composed of reticulated, sometimes atrophic white macules adjacent to erythema or telangiectasias

Red on white

The thin skin along the _____ is a frequent place to visualize the "red-on-white" pattern

Bitemporal hairline

This morphology is a very useful diagnostic tool because it does not seem to be associated with other connective tissue diseases

Red on white

Atrophy, hypopigmentation, hyperpigmentation, and telangiectasias

Poikiloderma

_____, as opposed to the "red-on-white", is a late manifestation and is not diagnostically specific

Poikiloderma

Typically a late manifestation in the skin, subcutaneous tissue, fascia, or muscle and typically affects the trunk proximal extremities, or areas of previous disease activity

Calcinosis

Calcinosis occurs more rapidly after disease onset in (juvenile/adult) versus (juvenile/adult) DM

Juvenile | Adult

Calcinosis is most frequent on the _____ in DM, in contrast to systemic sclerosis in which _____ calcinosis is most frequent

Extremities | Digital

In both juvenile and adult DM, the presence of _____ antibodies is associated with an increased risk of calcinosis

Anti-nuclear matrix protein 2 (NXP-2)

Calcinosis is also commonly seen in the _____ subset, which as associated with known vasculopathy

Anti-MDA5

Reflects active disease in DM, typically affecting the buttocks, trunk, and proximal extremities May progress to calcinosis or lipoatrophy

Panniculitis

Histopathology shows a _____ panniculitus but may have features of lupus panniculitis with lipomembranous changes or with septal thickening as in deep morphea

Lobular

Patients with _____ antibodies have a higher risk of alopecia, which commonly is severe and occurs early in the disease

Anti-MDA5

Has been recently found to be associated with anti-NXP2 antibodies and may predict more severe muscle disease

Subcutaneous edema

Most common pulmonary manifestation in DM and is a leading cause of morbidity and mortality

Interstitial lung disease

Pulmonary function tests show a

Restrictive disease pattern with a decreased forced vital capacity or a decreased diffusion capacity of carbon monoxide

Most common radiographic and histologic pattern in DM

Nonspecific interstitial pneumonia

Rare manifestation in DM | PFT revealing a disproportionately low diffusion capacity of carbon monoxide compared with a relatively normal FVC

Pulmonary arterial hypertension

Gold standard for the diagnosis of pulmonary arterial hypertension

Right heart catheterization

Myositis in DM typically presents as

Symmetrical proximal muscle weakness

Described as soreness or muscle tightness or burning, but muscles are not tender to palpation

Myalgia

Patients may note a hoarse or raspy voice (dysphonia) from _____ involvement

Cricoarytenoid muscle

May occur because of weak pharyngeal musculature

Dysphagia

Y/N: There is a high correlation between dysphagia and weakness of the anterior neck muscles

Yes

Distal muscle weakness in the hands, manifesting as difficulty opening jars or holding onto objects, more typically occurs late in disease or in patients with _____ antibodies

Anti-NXP2

Arthralgias are common in DM, reported in _____% of patients

30 to 40

Gastrointestinal involvement is an uncommon manifestation in juvenile DM, reported in _____% of juvenile patients with DM

Y/N: Cardiac involvement in DM is usually subclinical

Yes

May be a useful biomarker in detecting subclinical cardiac muscle involvement

Cardiac troponin I

DM is associated with malignancy in _____% of cases

10 to 20

Malignancies tend to occur within the first _____ of disease onset

1 to 2 years

Major antibody associated with malignancy

Anti-TIF1-gamma

DM is primarily an immune-mediated disorder with molecular and histologic evidence supporting a role for _____ immunity

Both innate and adaptive

Y/N: Myositis is not a requirement for the diagnosis of DM

Yes

A patient presenting with alopecia, cutaneous or mucosal ulceration, palmar erythematous papules, severe arthralgia or arthritis, and shortness of breath should trigger the clinician to consider

Anti-MDA5 DM

Patients with mechanic's hands, arthritis, Raynaud phenomenon, and lung symptoms are at high risk of having

Antisynthetase disease

Patients with extreme myalgia, peripheral edema, and distal weakness should be ruled out for DM specifically with

Anti-NXP2 antibodies

Myositis-specific autoantibodies associated with DM

``` TIF1-gamma NXP2 MDA5 SAE Mi-2 Jo-1 Other antisynthetase antibodies (PL-7, PL-12, EJ, OJ, SRP) ```

Early in the course of the disease, _____ are reasonably sensitive biomarkers of muscle inflammation. However, mid to late in the course of myositis, their sensitivity decreases

Serum muscle enzymes | Electromyographic studies

Serum muscle enzymes

``` Creatine kinase Aldolase Lactate dehydrogenase Aspartate aminotransferase Alanine aminotransferase ```

To increase the sensitivity of detecting myositis in laboratory testing, the clinician should

Evaluate creatine kinase, aldolase, and LDH as a group

All muscle enzymes can be elevated after strenuous activity; in questionable cases, the enzyme levels can be rechecked after

10-14 days following the activity

Can be elevated with liver disease or hemolysis of the blood sample

Aldolase | AST and ALT

Will be elevated with hepatic injury but will not be elevated in myositis Helpful addition to laboratory testing for myositis if hepatic sources are suspected

Gamma-glutamyl transferas

Serum ferritin is often highly elevated (>500 mg/dL) in _____ patients

Anti-MDA5

Sensitive indicator of myositis on MRI and correlates with creatine kinase levels

Muscle edema

If a muscle biopsy is necessary to confirm the diagnosis of DM, it has the highest yielded if performed within _____ of beginning any immunomodulatory therapy

2 weeks

Most common source of persistent disease skin activity

Skin over muscles or other organs

Y/N: Cutaneous DM often has discordant treatment response with the muscle disease, with recalcitrant skin disease continuing years after the muscle disease is in remission

Yes

In addition to a complete history and physical examination, routine age-appropriate cancer screening studies (_____), and relevant screening blood work (_____) as well as a urinalysis are indicated

Colonoscopy, mammogram, prostate examination | Complete blood count, renal and liver function tests

The manual muscle testing for a subset of eight muscle groups may be performed, which is a validated muscle test in which eight major muscle groups (_____) that are the highest yield in idiopathic inflammatory myopathies

``` Neck flexors Deltoids Biceps Wrist extensors Gluteus maximus Gluteus medius Quadriceps Ankle dorsiflexors ```

Serum muscle enzymes are checked _____ as biomarkers or myositis

At each visit

Found in both cardiac as well as regenerating skeletal muscle, so it is not a specific test, although it could be used as a screening test for cardiac involvement

Creatine kinase-MB

First line treatment of skin disease in DM

Photoprotection Topical steroids Hydroxychloroquine or chloroquine Quinacrine

First line treatment of muscle disease in DM

Systemic corticosteroids

Second line treatment of skin and muscle disease in DM

Methotrexate Mycophenolate mofetil IVIg Azathioprine

Although photoprotection is a key first step in management, up to _____% of patients with DM are actually minimally photosensitive, and as few as _____% may report disease exacerbation after UV exposure

60 | 20

First line treatment for myositis in combination with prednisone

Methotrexate

In patients with DM with suspected or diagnosed ILD, it is prudent to select a different agent than _____ because of its potential to induce acute pneumonitis and pulmonary fibrosis, thereby complicating the evaluation and management of ILD

Methotrexate

First-line oral agent when ILD is present

Mycophenolate mofetil

Most likely the single most effective agent for cutaneous DM

Intravenous immunoglobulin

Commonly used as maintenance therapy in the treatment of ILD associated with idiopathic inflammatory myopathies, typically after induction with cyclophosphamide

Azathioprine

Calcinosis remains one of the most formidable therapeutic challenges in the DM. _____ for localized lesions remains the most effective and definitive therapy

Surgical excision