Scleroderma Flashcards
What is scleroderma?
Acquired rheumatic disease with systemic sclerosis
What is the clinical presentation of scleroderma?
Thickening of skin with internal organ involvement
What is the cause of scleroderma?
No known cause
What are the 3 cardinal processes of scleroderma?
- Autoimmunity and inflammation
- Vascular injury and obliteration
- Fibrosis and matrix deposition
What is the path of scleroderma?
Capillary loss
Obliterative vasculopathy
- Intimal proliferation in small and medium vessels
Acellular fibrosis
- Deposition of connective tissue matrix
What tissues will be biopsied in scleroderma?
Skin
Lungs
GI
What will be found in a lung tissue biopsy?
Thickened alveolar septae
- Honeycombing
What will be found in a GI tissue biopsy?
- Fibrosis of lamina propria and submucosa
- Muscle atrophy
What will be found in a skin tissue biopsy?
- Collagen deposits in hair follicles
- Atrophic epidermis
What are the classifications of scleroderma?
- Systemic Sclerosis
- Mixed Connective Tissue Disorder
- Localized Scleroderma
What is involved with systemic scleroderma?
Diffuse cutaneous
Limited cutaneous
What is diffuse cutaneous?
Involves skin
- proximal to elbows and knees
- trunk
- distal extremities
Rapid progression
Early pulmonary fibrosis and acute renal failure
What is limited cutaneous?
Involves
- fingers
- toes
- distal extremities
- face
Trunk is spared
Raynaud’s phenomenon
What is CREST syndrome?
Calcinosis cutis Raynaud’s phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia
What is CREST syndrome involved with?
Limited cutaneous
What is localized scleroderma?
- Benign skin conditions
- Commonly affects children
- Morphea- reddish/purple lesions of the skin
- Skin induration
- No Raynaud’s phenomenon
- No systemic involvement
What is scleroderma attributed to?
Environmental factors
Genetics
What are the environmental factors of scleroderma?
Infectious agents
- Cytomegalovirus
- Parvovirus B19
Occupational
- Silica
- Epoxy resins
Dietary
Medications
- Bleomycin
- HRT
- Cocaine
- Appetite suppressants
Lifestyle exposures
What is involved in genetics of scleroderma?
First degree= higher risk
What is the initial presentation of diffuse cutaneous?
Soft tissue swelling Erythema Pruritus Fatigue Stiffness Malaise Raynaud’s will develop later
What is the initial presentation of limited cutaneous?
History of Raynaud’s
+/- ischemic ulcerations
Indolent and delayed onset of other signs (CREST syndrome)
Vascular manifestations
- Digital ischemia
- Telangiectasia
- Pulmonary HTN
What are the organs involved in scleroderma?
Skin GI tract Lungs Kidneys Cardiac MSK
What are the other manifestations of scleroderma?
Dry eyes Dry mouth Hypothyroidism CNS is spared usually - Trigeminal nerve fibrosis can occur
What labs are used in scleroderma
CBC with diff - Anemia Sed Rate - Normal CRP - Normal RA - Negative ANAs Vitamin deficiencies - Folate - Vit B12 - Vit D
What is nailfold capillaroscopy?
Differentiates between primary vs secondary raynaud’s
What is the diagnosis of scleroderma?
Skin induration
Raynaud’s phenomenon
Organ manifestations
What are the treatment options of scleroderma?
- No known regime to significantly alter the course
- Early recognition
- Accurate classification
- Risk stratification
- Early recognition of organ involvement
What immunosuppressive agents are used in treatment of scleroderma?
Corticosteroids
- Treats stiffness and aching
- Use sparingly and at low doses
Cyclophosphamide
- Decreased progression of symptomatic interstitial lung disease
- Weigh risks and benefits
Methotrexate
- Skin manifestations
What antibiotic theraphy is used in treatment of scleroderma?
D-Penicillamine
- Improves skin induration
- Prevents new internal organ involvement
- Improved survival
What has the worse prognosis?
Diffuse cutaneous systemic sclerosis
What is involved 2-4 years after onset of scleroderma?
Inflammatory symptoms- fatigue; edema, arthralgia- subside
Skin thickening plateau
Organ involvement- most rapid progression
When does scleroderma renal crisis occur?
Within 1st 4 years of onset
What are late manifestations of scleroderma?
PAH
GERD
What is involved in 10 year survival of scleroderma?
Diffuse cutaneous systemic scleroderma- 55%
Limited cutaneous systemic scleroderma- 75%
What are the leading causes of death in scleroderma?
Pulmonary fibrosis
PAH
GI involvement
Cardiac disease
What are some things that lead to a poor prognosis?
Male gender
AA race
Older age of disease onset
Low body mass index
Extensive skin thickening with truncal involvement
Presence of antibodies topoisomerase-I or RNA polymerase III
Absence of anticentromere antibodies
