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Fitzpatrick Tables > Chapter 63 - Systemic Sclerosis > Flashcards

Chapter 63 - Systemic Sclerosis Flashcards

(31 cards)

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1
Q

TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis

REACTIVITY

CLINICAL ASSOCIATION
Limited skin sclerosis, severe gut disease, isolated PAH, calcinosis

A

Centromere

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2
Q

TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis

REACTIVITY

CLINICAL ASSOCIATION
Diffuse skin sclerosis, pulmonary fibrosis and secondary PAH, increased SSc-related mortality rate

A

Scl-70

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3
Q

TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis

REACTIVITY

CLINICAL ASSOCIATION
Diffuse skin sclerosis, hypertensive renal crisis, correlated
with a higher mortality rate

A

RNAP III

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4
Q

TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis

REACTIVITY

CLINICAL ASSOCIATION
Overlap features of SLE, arthritis

A

nRNP

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5
Q

TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis

REACTIVITY

CLINICAL ASSOCIATION
Limited skin sclerosis, myositis–sclerosis overlap, calcinosis

A

Pm-Scl

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6
Q

TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis

REACTIVITY

CLINICAL ASSOCIATION
Diffuse skin sclerosis, myositis, PAH, renal disease

A

Fibrillarin

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7
Q

TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis

REACTIVITY

CLINICAL ASSOCIATION
Limited skin sclerosis, pulmonary fibrosis

A

Th/To

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8
Q

TABLE 63-4
Functional Autoantibodies in Systemic Sclerosis and Their Association to Pathophysiology

FUNCTIONAL ANTIBODY

CLINICAL ASSOCIATION
- Seem to induce skin fibrosis as a result of activation of fibroblasts into myofibroblasts and fibroblast-like cells
- First functional antibodies discovered in systemic sclerosis (SSc)

A

Anti–platelet-derived growth factor receptor

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9
Q

TABLE 63-4
Functional Autoantibodies in Systemic Sclerosis and Their Association to Pathophysiology

FUNCTIONAL ANTIBODY

CLINICAL ASSOCIATION
- Mediates endothelial cell damage and activation of fibroblasts resulting from stimulation of proinflammatory and fibrotic cytokines
- Associated with severe organ manifestation
- Associated with perivascular, vascular (digital ulcers [DUs]) and lung involvement (pulmonary arterial hypertension [PAH])
- Also found in other rheumatic diseases

A

Antiendothelial cell antibodies

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10
Q

TABLE 63-4
Functional Autoantibodies in Systemic Sclerosis and Their Association to Pathophysiology

FUNCTIONAL ANTIBODY

CLINICAL ASSOCIATION
- Associated with Scl-70 antibodies and the prevalence of interstitial lung disease and PAH
- Increased prevalence in diffuse cutaneous SSc compared to limited cutaneous SSc

A

Antifibroblast antibodies

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11
Q

TABLE 63-4
Functional Autoantibodies in Systemic Sclerosis and Their Association to Pathophysiology

FUNCTIONAL ANTIBODY

CLINICAL ASSOCIATION
- Activates fibroblasts by stimulation of the release of transforming growth factor-ββ

A

Antifibrillin-1

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12
Q

TABLE 63-4
Functional Autoantibodies in Systemic Sclerosis and Their Association to Pathophysiology

FUNCTIONAL ANTIBODY

CLINICAL ASSOCIATION
- Inhibits the degradation of extracellular matrix proteins, because of an inhibition of MMP collagenase activity
- Correlates with the extent of fibrosis (skin, lung, kidney)

A

Anti–matrix metalloproteinase (MMP) 1, anti-MMP3

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13
Q

TABLE 63-4
Functional Autoantibodies in Systemic Sclerosis and Their Association to Pathophysiology

FUNCTIONAL ANTIBODY

CLINICAL ASSOCIATION
- Simultaneous presence has been described in SSc patients (cross-reactivity)
- Associated with early and severe disease, PAH, DUs, renal crisis, diffuse cutaneous SSc, and lung fibrosis

A

Angiotensin II Type 1 receptor and endothelin Type A receptor

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14
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Raynaud phenomenon (4)
(Vasculopathy)

A
  1. Consistent warm keeping, paraffin-bath, patient education
  2. Calcium channel blockers (eg, nifedipine) by mouth
  3. Angiotensin receptor antagonists
  4. Alternatives: selective serotonin reuptake inhibitors (SSRIs), α-blockers, sympathectomy with or without botulinum toxin injection
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15
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Digital ulcers (4)
(Vasculopathy)

A
  1. Prostacyclin (eg, iloprost) IV
  2. Endothelin receptor blockade (eg, bosentan by mouth)
  3. Phosphodiesterase Type 5 inhibitors (off-label)
  4. Wound dressing (hydrocolloid membrane, Mepilex)
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16
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Synovitis/myositis (2)
(Musculoskeletal system)

Study These Flashcards
A
  1. Methotrexate (by mouth, IM)
  2. Rituximab (off-label)
17
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Reflux (2)
(GI tract)

Study These Flashcards
A
  1. Proton pump inhibitors
  2. Prokinetics
18
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Dysphagia (1)
(GI tract)

Study These Flashcards
A
  1. H2-receptor antagonists
19
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Diarrhea, obstipation (3)
(GI tract)

Study These Flashcards
A
  1. Change habit of eating, parenteral nutrition
  2. Antibiotics (eg, ciprofloxacin)
  3. Symptomatic management with antidiarrheal agents or laxatives
20
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Dyspnea (1)
(Respiratory system)

Study These Flashcards
A
  1. Oxygen, if necessary
21
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Alveolitis/lung fibrosis (3)
(Respiratory system)

Study These Flashcards
A
  1. Cyclophosphamide IV
  2. Mycophenolate mofetil by mouth (used as an alternative or after cyclophosphamide)
  3. Glucocorticoids (short dated, if necessary)
22
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Pulmonary arterial hypertension (7)
(Cardiac system)

Study These Flashcards
A
  1. Oxygen, if necessary
  2. Diuretics
  3. Endothelin receptor blockade (eg, bosentan by mouth, macitentan)
  4. Inhaled iloprost
  5. Phosphodiesterase Type 5 inhibitors (eg, sildenafil by mouth, tadalafil)
  6. Epoprostenol by mouth
  7. Combination of different agents
23
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Systolic heart failures (3)
(Cardiac system)

Study These Flashcards
A
  1. Immunosuppression with or without pacemaker
  2. Cardioverter defibrillator
  3. Angiotensin-converting enzyme inhibitors and carvedilol (selective β-blockers may be considered, but consider worsening of Raynaud phenomenon)
24
Q

TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis

Diastolic heart failure (2)
(Cardiac system)

Study These Flashcards
A
  1. Diuretics
  2. Calcium channel inhibitors
25
TABLE 63-7 Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis Scleroderma renal crisis (1) (Kidney)
1. Angiotensin-converting enzyme–Hemmer (high-dosed)
26
TABLE 63-8 Therapeutic Options for Skin Involvement in Systemic Sclerosis Skin hardening (5)
1. Lymphatic drainage 2. Physiotherapy 3. Topical treatment with steroids or calcineurin inhibitors 4. Systemic treatment with steroids (short dated) and/or immunosuppressants 5. Phototherapy (psoralen and ultraviolet A, ultraviolet A1, extracorporeal photochemotherapy)
27
TABLE 63-8 Therapeutic Options for Skin Involvement in Systemic Sclerosis Dryness and itching (5)
1. Topical treatment with steroids, capsaicin 2. Cannabinoid agonists 3. Emollients 4. Phototherapy (psoralen and ultraviolet A, ultraviolet A1) 5. Systemic treatment with antihistamines or gabapentin
28
TABLE 63-8 Therapeutic Options for Skin Involvement in Systemic Sclerosis Digital ulcerations (5)
1. IV iloprost 2. Bosentan by mouth 3. Hydrocolloid dressings 4. Skin substitutes 5. Physical therapy
29
TABLE 63-8 Therapeutic Options for Skin Involvement in Systemic Sclerosis Calcifications (4)
1. Bisphosphonate by mouth 2. Local corticosteroid injection 3. Laser therapy 4. Surgery
30
TABLE 63-8 Therapeutic Options for Skin Involvement in Systemic Sclerosis Telangiectases (2)
1. Laser therapy 2. Camouflage
31
TABLE 63-8 Therapeutic Options for Skin Involvement in Systemic Sclerosis Hyperpigmentation and hypopigmentation (3)
1. Bleaching agents, camouflage, sunscreens 2. Salicylic acid and chemical peelings 3. Hydroquinone, retinoids, corticosteroids

Fitzpatrick Tables (92 decks)

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