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College Learning Series > Systemic Sclerosis and Inflammatory myopathies > Flashcards

Systemic Sclerosis and Inflammatory myopathies Flashcards

1
Q

Systemic Sclerosis gender prediliciton, age group

A

F:M = 3:1

Peak 40-60yo

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2
Q

Risk factors for systemic sclerosis

A

HLA-DRB1, STAT4, interferon genes

Silica, organic solvents, vinyl chloride

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3
Q

Diffuse Vs Limited Systemic Sclerosis

A

In diffuse:
- Raynauds Shorter Hx
- Rapid onset skin changes, contracture
- More constitutional Symptoms
Early onset Internal organ involvement (ILD, renal crisis)

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4
Q

Serum Abs in Systemic Sclerosis

A

Scl-70 (topoisomerase 1) -> ILD

Centromere -> protection from ILD/ renal disease, much more common in limited

RNA Polymerase III -> renal, skin involvement, malignancy

U1-RNP - overlap features

Th/ To - PAH

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5
Q

Average decrease life span with scleroderma?

A

23 years of life lost in women and 26 years of life lost in men

Highest mortality among the rheumatic diseases.

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6
Q

Management of Systemic sclerosis

A

Often symptomatic relief only

Monitor for new organ involvement
- ILD or renal disease first 5 years dcSSc (4-6 monthly HRCT)
- PHTN - anytime, both groups (BNP, PFTs, ? TTE +/- RHC if sus)

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7
Q

Radiological pattern of Pulm HTN in Systemic Sclerosis

A

NSIP > UIP

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8
Q

Predictors of worsening Systemic Sclerosis?

A

Male,
African American
dcSSc

biomarkers - SCL70 Ab, etc etc

Pulm HTN
- FVC < 70,
- DLCO < 55%,
- HRCT fibrosis bad

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9
Q

Systemic Sclerosis ILD management?

A

PPIs, quit smoking, vaccinate, O2 as needed

1st line: mycophenelate

2nd line: cyclophosphamide, followed by MMF or azathioprine

3rd line: ?nintendandib (good new evidence for)/ pirfenidone/ rituximab/ tocilizumab/ lung transplant/ stem cell transplant

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10
Q

Pulm HTN Group in Systemic Sclerosis

A

Group 1 - pre-capillary

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11
Q

Difference vs pre/ post capillary pull HTN on RHC?

A

Pre:
- PAWP <= 15mmHg,
- WU >= 3

Post:
- PAWP > 15
- WU < 3

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12
Q

Treatment of Pulm HTN in Systemic Sclerosis

A

Use meds based on severity
- no real role CCBs
- endothelial receptor antagonists (ambrisentan, bosentan, macitentan),
- PDE-5 inhibitors (sildenafil, tadalafil)
- rociguat
- if severe: IV epoprostenol
- ? prostacyclin analogues (iloprost, treprostinil)

RCTs included patients with CTD-PAH but the effects of PAH specific therapies were not as significant as for iPAH

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13
Q

Clinical features Scleroderma Renal Crisis?

A
  • Abrupt onset of moderate to severe hypertension
  • Progressive renal failure
  • Normal urine sediment or only mild proteinuria with few cells or casts

Signs of more severe SRC (Reflect underlying vasculopath/ HTN)
* MAHA/ thrombocytopenia
* Heart failure/ flash pulmonary oedema
* Blurred vision due to retinopathy
* Headache, fever, malaise
* Encephalopathy - may be complicated by generalized seizures
* Pericardial effusion

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14
Q

Risk Factors for Scleroderma Renal Crisis?

A
  • diffuse SSc (10-20%),
  • RNA polymerase III Ab,
  • tendon friction rubs
  • corticosteroids
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15
Q

Management of scleroderma renal crisis?

A

BP control
- ACEi
- IV nitroprusside (CNS involved)

Consider ICU
Wean/ Cease steroids
Dialysis
? renal transplant

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16
Q

Prognostic factors scleroderma renal crisis?

A

Worse in:
- males
- not that hypertensive initially
- already on ACEi

17
Q

Treatment for Raynaud’s?

A

Treat underlying cause

1st line: CCB

2nd line: ARB/ PDE5i/ nitrates/ alpha blockers/ SSRI/ anti-platelet/ statin

3rd line: IV prostacyclins botox

18
Q

4 Main types Inflammatory Myopathy?

A
  • Polymyositis (PM)
  • Dermatomyositis, including amyopathic DM
  • Inclusion body myositis (IBM)
  • Immune-mediated necrotising myopathy (IMNM) * (+overlap myositis)

Characterized by the shared features of proximal skeletal muscle weakness and evidence of immune-mediated muscle inflammation and distinct clinical, serological, histopathological features

19
Q

Pathophys of Inflammatory Myopathies

A
20
Q

Clinical features of Dermatomyositis/ Polymyositis?

A

Symmetrical muscle weakness (90%)
- w myalgia/ tenderness
- delts + neck/ hips (flexors)
- diaphragm, intercostal muscles -> hypoventilation

Rash
- heliotrope rash
- shawl sign (poikiloderma)
- Gottron’s papules/ sign

Myocarditis +/- MI

ILD (10%)

21
Q

Ix in PM/ DM

A

Elevated muscle enzymes: CK/ transaminases/ LDH/ aldolase
- 10x ULN
- may not correlate well with weakness

ANAs
- myositis specific (30-40%) or myositis associated

EMG - differentiate from neuropathy

MRI

Muscle biopsy - good to have MRI b4 to target

22
Q

Anti-synthetase syndrome

A
  • up to 30% PM and DM
  • mechanic’s hands
  • constitutional symptoms
  • myositis
  • Raynaud phenomenon
  • non-erosive arthritis
  • ILD
  • Abs to aminoacyl-transfer ribonucleic acid
    (tRNA) synthetase enzymes
23
Q

Clinically Amyopathic Dermatomyositis (CADM)

A
  • 10-30% of DM
  • classic cutaneous features of DM without muscle
    involvement
  • increased risk of malignancy (<DM)
  • may be more likely if also MDA5+ve
24
Q

Investigations for DM/ PM Complications

A

Malignancy
- age appropriate screening
- physical exams
- lab testing (???)

Cardiac
- TTE/ ECG

Pulm:
- PFTs, HRCT

Oesophageal dysfunction
- oesophageal dysmotility studies

25
Q

Treatment for DM/ PM/ IMNM

A

High dose glucocorticoids tapering over one year
* 1mg/kg/dupto80mg/d
* IV methyl prednisolone 10000mg x 3 if severe

Steroid-sparing agents
* Azathioprine
* Methotrexate
* IVIg
* Rituximab
* Plasma exchange

Monitor: CK, muscle power, PFT

Other measures:
* Monitor respiratory function, measures to reduce aspiration, exercise, photoprotection if skin disease, prevention osteoporosis and infection (vaccines, Bactrim)

26
Q

Inclusion Body Myositis

A
  • Insidious onset of asymmetric, proximal leg weakness for up to 5 years
  • Weakness of distal finger flexor muscles in 95%
  • More muscle atrophy
  • Dysphagia due to cricopharyngeal muscle involvement in ~ 1/3 to 1⁄2
  • CK elevated < 10 x ULN, not useful for monitoring disease, ESR, CRP usually normal
  • 15% have an autoimmune disease
  • EMG, MRI, muscle biopsy
  • Less responsive to immunosuppressive therapy - A limited trial of GCs

College Learning Series (11 decks)

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