Scleroderma Flashcards
Key Points:
•Initial pathogenesis of scleroderma is mostly unknown but does include 3?
•Vascular manifestations typically precede ??? manifestations.
•Classification includes ? and ? manifestations.
•Systemic disease is further classified into ? and ?.
•Diagnosis is based on clinical symptoms with ? lab testing.
•Prognosis and conventional treatment depends on ? involvement.
•Treatment should focus on ?and ? treatments.
Key Points:
•Initial pathogenesis of scleroderma is mostly unknown but does include immune dysfunction, microangiopathy and systemic fibrosis.
•Vascular manifestations typically precede fibrotic manifestations.
•Classification includes localized and systemic manifestations.
•Systemic disease is further classified into limited and diffuse. (Know the difference)
•Diagnosis is based on clinical symptoms with serologic lab testing.
•Prognosis and conventional treatment depends on organ involvement.
•Treatment should focus on anti-inflammatory and anti-oxidant treatments.
Epidemiology:
•Much more common in ? (5x)
•Limited and diffuse scleroderma is higher in ? women
•Localized scleroderma is higher in ? women
•Familial links are thought to be more associated with ? factors than genetic ones.
Epidemiology:
•Much more common in women (5x)
•Limited and diffuse scleroderma is higher in African American women
•Localized scleroderma is higher in Caucasian women
•Familial links are thought to be more associated with environmental factors than genetic ones.
Etiology: 3?
The etiology of scleroderma is mostly unknown.
Etiology:
The etiology of scleroderma is mostly unknown.
- Genetics
- Environmental Triggers
- Infections
Clinical Features: 5
Systemic symptoms:
Musculoskeletal symptoms:
Skin Lesions:
Vascular Symptoms:
Organ Involvement
Clinical Features:
Systemic symptoms: 5
Systemic symptoms: •Fatigue •Stiff joints (arthralgias) •Myalgias •Loss of muscle strength •Sleep difficulties
Clinical Features:
Musculoskeletal symptoms: 3
Musculoskeletal symptoms:
•Joint swelling/pain
•Immobility
•Nerve entrapment
Clinical Features:
Skin Lesions: 5
Skin Lesions:
•Early stages: pruritic lesions, edema, hyper/depigmentation
•Thickening of skin, decreased pliability, fixation other structures.
•Sclerodactyly
•Telangiectasias
•Calcinosis cutis
Lesion distribution will determine classification.
Clinical Features: 3
Vascular Symptoms:
Vascular Symptoms:
•Raynaud phenomenon – color changes in the digits from cold/stress
•Color changes: white, blue (acrocyanosis), red (reperfusion hyperemia)
•Telangiectasias
Clinical Features:
Organ Involvement:
Clinical Features:
Organ Involvement
•GI: esophageal hypomobility, GERD, esophagitis, Barrett’s esophagus
•Pulmonary: Interstitial lung disease, pulmonary vascular disease
•Renal: renal failure
•Renal crisis – acute renal failure in 10 – 15% of patients
•Cardiac: Pericarditis, pericardial effusions, myocardial fibrosis
Classifications:
Localized- 2?
Systemic – defined by extent of skin and organ involvement
•Diffuse Cutaneous ??
•Limited Cutaneous – restricted skin sclerosis to hands, face, neck
•Prominent vascular symptoms and CREST syndrome
Classifications:
Localized
1. •Linear – skin and subcutaneous tissues following a dermatome
2. •Morphea – patches of sclerotic skin on the trunk and limbs
Systemic – defined by extent of skin and organ involvement
1. •Diffuse Cutaneous – extensive skin sclerosis – trunk, limbs
•At greater risk for developing renal, lung and cardiac disease
•(W/up every yr at 1st - pulmonary/cardiac, then every several yrs.)
2. •Limited Cutaneous – restricted skin sclerosis to hands, face, neck
•Prominent vascular symptoms and CREST syndrome
Laboratory Testing:
Serologic Testing
•Anti-nuclear antibodies – present in ?% of patients with SSc
•Antitopoisomerase I (anti-Scl-70) – associated with ?cSSc
•Anticentromere antibodies (ACA) – associated with ?cSSc
•Anti-RNA polymerase III antibodies – associated w/?cSSc and ? and ?
Laboratory Testing:
Serologic Testing
•Anti-nuclear antibodies – present in 95% of patients with SSc
•Antitopoisomerase I (anti-Scl-70) – associated with dcSSc
•Anticentromere antibodies (ACA) – associated with lcSSc
•Anti-RNA polymerase III antibodies – associated with dcSSc and rapidly progressive skin involvement and renal crisis
Follow-Up Testing: 5
Follow-Up Testing:
The following tests should be ordered to monitor further complications
1. •CBC
•Rule out anemia from GI blood loss, iron deficiency or malabsorption
2. •Chem Panel
•Creatinine – assess renal dysfunction
3. •Urinalysis – Important!
•Assess for hematuria, proteinuria or casts
4. •Creatinine kinase (CK)
•Elevated in myopathy or myositis
5•Chest X-ray
•All patients with SSc should be evaluated for interstitial lung disease!
Conventional Medications: 4
Conventional Medications:
Treatment depends on extent of organ involvement.
Severe organ involvement – immunosuppressive therapy
1. •Cyclophosphamide – antineoplastic, antirheumatic, immunosuppressive; controversial as controlled studies not done – the main choice if there is pulmonary involvement
2. •Glucocorticoids – not generally recommended due to renal crisis
3. •Cyclosporine – suppresses T cells and reduces collagen synthesis – also nephrotoxic – best for improving skin tightness
Antifibrotics (slow acting and not strong enough for severe fibrosis)
4. •D-penicillamine – metal chelator that affects collagen biosynthesis
Dietary Modifications: 9
Dietary Modifications:
- •Modifications for dysbiosis: low carbohydrate, low sugar
- •Care with increasing fiber in diet18
- •Anti-inflammatory diet
- •reduction in red meat, dairy, sugar, carbohydrates, saturated fats
- •Activate TLR, NF-kappa B, increase oxidative stress
- •Promote foods high in vitamins and minerals – malabsorption is common
- •Elimination of food allergens
- •Gluten19,20
- •Short-term fasting
Viral Infections: 4
Viral Infections:
•Viruses associated with scleroderma: CMV, parvovirus.
•Andrographis (Chuan Xin Lian) – SE with 30mg of andrographolides TID x 2-3 weeks
•Monolaurin – Antiviral properties against CMV – 600mg TID
•Reishi –(Ganoderma lucidum) – 2-6g of raw reishi or equivalent of concentrated extract.
