Chapter 63 - Systemic Sclerosis Flashcards
TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis
REACTIVITY
CLINICAL ASSOCIATION
Limited skin sclerosis, severe gut disease, isolated PAH, calcinosis
Centromere
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
Scl-70
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
RNAP III
TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis
REACTIVITY
CLINICAL ASSOCIATION
Overlap features of SLE, arthritis
nRNP
TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis
REACTIVITY
CLINICAL ASSOCIATION
Limited skin sclerosis, myositis–sclerosis overlap, calcinosis
Pm-Scl
TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis
REACTIVITY
CLINICAL ASSOCIATION
Diffuse skin sclerosis, myositis, PAH, renal disease
Fibrillarin
TABLE 63-3
Clinical Association of Hallmark Autoantibodies in Systemic Sclerosis
REACTIVITY
CLINICAL ASSOCIATION
Limited skin sclerosis, pulmonary fibrosis
Th/To
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)
Anti–platelet-derived growth factor receptor
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
Antiendothelial cell antibodies
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
Antifibroblast antibodies
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
Antifibrillin-1
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)
Anti–matrix metalloproteinase (MMP) 1, anti-MMP3
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
Angiotensin II Type 1 receptor and endothelin Type A receptor
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Raynaud phenomenon (4)
(Vasculopathy)
- Consistent warm keeping, paraffin-bath, patient education
- Calcium channel blockers (eg, nifedipine) by mouth
- Angiotensin receptor antagonists
- Alternatives: selective serotonin reuptake inhibitors (SSRIs), α-blockers, sympathectomy with or without botulinum toxin injection
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Digital ulcers (4)
(Vasculopathy)
- Prostacyclin (eg, iloprost) IV
- Endothelin receptor blockade (eg, bosentan by mouth)
- Phosphodiesterase Type 5 inhibitors (off-label)
- Wound dressing (hydrocolloid membrane, Mepilex)
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Synovitis/myositis (2)
(Musculoskeletal system)
- Methotrexate (by mouth, IM)
- Rituximab (off-label)
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Reflux (2)
(GI tract)
- Proton pump inhibitors
- Prokinetics
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Dysphagia (1)
(GI tract)
- H2-receptor antagonists
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Diarrhea, obstipation (3)
(GI tract)
- Change habit of eating, parenteral nutrition
- Antibiotics (eg, ciprofloxacin)
- Symptomatic management with antidiarrheal agents or laxatives
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Dyspnea (1)
(Respiratory system)
- Oxygen, if necessary
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Alveolitis/lung fibrosis (3)
(Respiratory system)
- Cyclophosphamide IV
- Mycophenolate mofetil by mouth (used as an alternative or after cyclophosphamide)
- Glucocorticoids (short dated, if necessary)
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Pulmonary arterial hypertension (7)
(Cardiac system)
- Oxygen, if necessary
- Diuretics
- Endothelin receptor blockade (eg, bosentan by mouth, macitentan)
- Inhaled iloprost
- Phosphodiesterase Type 5 inhibitors (eg, sildenafil by mouth, tadalafil)
- Epoprostenol by mouth
- Combination of different agents
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Systolic heart failures (3)
(Cardiac system)
- Immunosuppression with or without pacemaker
- Cardioverter defibrillator
- Angiotensin-converting enzyme inhibitors and carvedilol (selective β-blockers may be considered, but consider worsening of Raynaud phenomenon)
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Diastolic heart failure (2)
(Cardiac system)
- Diuretics
- Calcium channel inhibitors
TABLE 63-7
Recommended Therapeutic Strategies for Internal Organ Involvement in Systemic Sclerosis
Scleroderma renal crisis (1)
(Kidney)
- Angiotensin-converting enzyme–Hemmer (high-dosed)
TABLE 63-8
Therapeutic Options for Skin Involvement in Systemic Sclerosis
Skin hardening (5)
- Lymphatic drainage
- Physiotherapy
- Topical treatment with steroids or calcineurin inhibitors
- Systemic treatment with steroids (short dated) and/or immunosuppressants
- Phototherapy (psoralen and ultraviolet A, ultraviolet A1, extracorporeal photochemotherapy)
TABLE 63-8
Therapeutic Options for Skin Involvement in Systemic Sclerosis
Dryness and itching (5)
- Topical treatment with steroids, capsaicin
- Cannabinoid agonists
- Emollients
- Phototherapy (psoralen and ultraviolet A, ultraviolet A1)
- Systemic treatment with antihistamines or gabapentin
TABLE 63-8
Therapeutic Options for Skin Involvement in Systemic Sclerosis
Digital ulcerations (5)
- IV iloprost
- Bosentan by mouth
- Hydrocolloid dressings
- Skin substitutes
- Physical therapy
TABLE 63-8
Therapeutic Options for Skin Involvement in Systemic Sclerosis
Calcifications (4)
- Bisphosphonate by mouth
- Local corticosteroid injection
- Laser therapy
- Surgery
TABLE 63-8
Therapeutic Options for Skin Involvement in Systemic Sclerosis
Telangiectases (2)
- Laser therapy
- Camouflage
TABLE 63-8
Therapeutic Options for Skin Involvement in Systemic Sclerosis
Hyperpigmentation and hypopigmentation (3)
- Bleaching agents, camouflage, sunscreens
- Salicylic acid and chemical peelings
- Hydroquinone, retinoids, corticosteroids
