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Chapter 51 connective tissue diseases Melissa > Dermatomyositis > Flashcards

Dermatomyositis Flashcards

1
Q

Types of adult onset dermatomyositis

A 
Classic
Classic with malignancy
Classic with overlapping connective tissue disorder
Amyopathic  DM 
Hypomyopathic DM
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2
Q

Types of juvenile onset DM

A

Classic
Amyopathic DM
Hypomyopathic

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3
Q

Types of polymyositis

A

Isolated polymyositis
Polymyositis as part of an overlapping connective tissue disorder
Polymyoisitis associated with internal malignancy

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4
Q

Drugs associated with DM

A 
PHD TO BOOST (the) CV
Penicillamine, Hydroxyurea
Diclofenac, Tamoxifen
Benzalkonium Chloride
Carbamazepine
Vaccination (BCG)
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5
Q

Characteristics of amyopathic

A

1% amyopathic, 76% female

29% ILD (17% classic)

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6
Q

Anti-aminoacyl-tRNA synthetases: eg anti-Jo-1, antiPL-7
Anti-SRP
Anti-Mi-2

A

More common in adult onset classic

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7
Q

Abs in adult onset amyopathic

A

Anti-p155/140: 80%
Anti-SAE
Anti-CADM-140 (MDA5)- associated with ILD

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8
Q

Abs in juvenile disease

A 
Anti-aminoacyl-tRNA synthetases: 
eg anti-Jo-1, antiPL-7
Anti-SRP
Anti-Mi-2
Anti-p155/140
Anti-p140
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9
Q

Anti-SRP

A

Acute onset, necrotising myopathy (severe weakness, high CK), may be refractory to tx

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10
Q

AntiMi2

A

Milder disease, hallmark is cutaneous disease, milder muscle disease with good response to treatment

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11
Q

Antip155/140

A

More associated with cancer associated myositis, severe cutaneous disease and amyopathic disease

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12
Q

antip140

A

Associated with juvenile + calcinosis

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13
Q

anti-SAE

A

Adults, may present with amyopathic

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14
Q

Anti CADM140 (MDA5)

A

Clinically amyopathic

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15
Q

Types of malignancies

A

Mainly Carcinomas
Genitourinary malignancies esp ovarian, colon cancer
South east Asian populations: nasopharyngeal CA
Breast, lung, gastric, pancreatitic, lymphomas, female genital
Risk may return to normal after 2-5 years

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16
Q

What is antisynthetase syndrome

A

Antiaminoacyl-tRNA synthetases (eg antiJo1, threonyl): antisynthetase syndrome: myositis, mechanics hands, fever, raynauds, gottrons, arthritis, ILD.

17
Q

Investigations for DM (not myositis)

A

ANA, ENA, dsDNA, TSH, fbc, elfts- look for overlapping disease
Lung 15-30%: RFTs with diffusion capacity – screen yearly, Chest Xray, HRCT . Associated with antisynthetase syndrome, MDA5
Cardiac - arrythmias: ECG, if symptomatic Echo, holter
GIT: if symptoms: barium swallow
If planning CS: DEXA bone

18
Q

Investigations for myositis

A

Serum muscle enzyme levels – CK (95% at some point), aldolase, urine creatine, urine or serum myoglobulin
AST, ALT and LDH may be elevated in myositis

Electromyography (90% abnormal), less helpful in polymyositis as overlap with other dx

T2 weighted MRI of proximal limbs
Muscle bx - triceps

If amyopathic: examine for muscle weakness and enzymes 2-3/12 for 2 years

19
Q

Evaluation for malignancy

A 
Baseline and regularly for at least 2-5 years 
Both
Urinalysis, CT chest/abdo/elvis
FOBT or if Sx/anaemia 
Colonscopy 
Upper endoscopy 
Serum protein and immunofixation electrophoresis 
CA19.9
Males:PSA
Females: Mammogram, TV pelvic USS, CA125
20
Q

Common skin signs (9)

A

Heliotrope sign
Eyelid oedema
Gottron’s papules – flat topped lichenoid papules on PIP and MCP joints
Gottron’s sign – violaceous discoloration of knuckles, elbows or knees
Photodistributed poikiloderma (includes facial erythema, shawl sign)
Scalp poikiloderma
Non scarring alopecia
Calcinosis cutis (especially in juvenile dermatomyositis)- associated with delay in CS, or severe disease. May drain a chalky material via fistulae. Usually elbows, knees, fingers, may be painful (sites of trauma)
Nail fold changes (ragged cutis, nail fold telangiectasis – dilated capillary loops alternating with vessel dropout)

21
Q

Uncommon signs

A

Cutaneous erosions or ulceration
Holster sign (poikiloderma of the lateral thighs)
Flagellate erythema
Vesicobullous lesions
Efoliative erythroderma
Panniculitis
Gingival telangiectasis
Pustular eruption of the elbows and knees
Lipoatrophy (esp in juvenile)
Small vessel vasculitis (esp in juvenile)

22
Q

Poor prognostic features

A

Malignancy, Cardiac involvement, older age, progressive disease, initiation of therapy after 24 months of muscle weakness, longer duration of symptoms prior to Dx, pulmonary disease, dysphagia, extensive cutaneous involvement of the trunk.

23
Q

Good prognostic features

A

With no muscle involvement – better

Calcinosis is a good prognostic feature

24
Q

Mortality

A

1/4

25
Q

Death from?

A

Death usually from : CA, respiratory infection, cardiac failure, malnutrition, weakness due to difficulty in swallowing

26
Q

Cutaneous tx

A

Photoprotection (sunscreen including protection against UVA)
Topical CSA – particularly for pruritis
Topical Tacrolimus- particularly for pruritis
HCQ 200mg bd (increased frequency of drug eruptions in DM) – 1/3 have rash usually morbilliform, may tolerate switch to chloroquine
Low dose weekly MTX (5-15mg/week)
MMF
High dose IVIg (2g/kg/month) – then spread to 7 week 2g/kg over 2 days
Retinoids
Dapsone
Thalidomide
Leflunomide
Anti-oestrogens
TNF alpha – can cause DM
Rituximab
Tacrolimus

27
Q

Systemic treatment

A 
Oral pred 1mg/kg/day tapered to 50% over 6 months, then to zero over 2-3 years
MTX 5-20mg weekly
AZA 2-3mg/kg/day
High dose IVIg 2g/kg/month
Pulse cyclophosmaide 0.5-1g/m2
Chlorambucil
Cyclosporine 3-5mg/kg/day 
Tacrolimus
MMF 1g bd
Sirolimus 
Infliximab
Etanercept
Rituximab
Fludarabine
Haematopoietic stem cell transplant
Plasmaphoresis
28
Q

Ongoing monitoring

A

Regular muscle enzyme monitoring every 2-3 months for 2-3 years if amyopathic at baseline
Regular RFTS including diffusion capacity
Baseline: DEXA, lipids and fasting glucose if on oral pred (children esp: check developmental milestones, lipodystrophy, hypertrigs, insulin resistance)
Regular malignancy screening 4-6 months for 2-3 years
Physical therapy
Calcinosis cutis may respond to diltiazem and /or surgical excision
Not all derm disease will be treated with muscle disease remittance
Rare panniculitis may respond to HCQ

Chapter 51 connective tissue diseases Melissa (3 decks)

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