63 - Systemic Sclerosis Flashcards
(Women/Men) are more frequently affected by systemic sclerosis
Women
Age onset of systemic sclerosis
Between 30 and 50 years
Y/N: Systemic sclerosis has the highest case-specific mortality of any of the autoimmune rheumatic diseases
Yes
Systemic sclerosis usually starts with
Raynaud phenomenon
Hallmark complications of systemic sclerosis
Hypertensive scleroderma renal crisis
Pulmonary arterial hypertension
Pulmonary fibrosis
GI dysmotility
For the diagnosis of systemic sclerosis to be proven, the American College of Rheumatology classification (1980) requires
Either 1 major criterion or
At least 2 minor criteria
Major criterion according to the American College of Rheumatology classification (1980)
Scleroderma proximal to the metacarpophalangeal or metatarsophalangeal joints
Minor criteria according to the American College of Rheumatology classification (1980)
Sclerodactyly
Digital ulcerations and/or pitting digital scars
Bibasilar pulmonary fibrosis
The new ACR/European League Against Rheumatism criteria considered several additional criteria such as
Abnormal nailfold capillaries
Fingertip lesions
Autoantibodies
Defined as a progressive form of SSc with an early onset of RP, usually within 1 year of onset of skin thickening
Diffuse cutaneous SSc
Diffuse cutaneous SSc shows very freuqently _____ antibodies
Anti-scleroderma 70 (antitoposiomerase-I) or
Anti-RNA polymerase III
Diffuse cutaneous SSc has a higher propensity to develop
Pulmonary fibrosis
Cardiac involvement
Scleroderma renal crisis
Characterized by a long preexisting history of RP and skin changes of the extremities distal to the knee and elbow joints, including facial skin
Limited cutaneous SSc
Limited cutaneous SSc often presents with _____ antibodies
Anticentromere
Limited cutaneous SSc is frequently associated with
Isolated pulmonary arterial hypertension
CREST is a _____ form of SSc
Limited
CREST meaning
Calcinosis RP Esophageal dysmotility Sclerodactyly Telangiectasias
Defined by positive RP and at least 1 additional feature of SSc (positive nailfold capillary alterations, puffy fingers, pulmonary hypertension) and/or detectable scleroderma-associated autoantibodies without fulfilling the ACR criteria
Early or undifferentiated SSc
Develop vascular (RP and/or PAH), immunologic (most commonly anticentromere antibodies), and organ-based fibrotic features of SSc, but do not show skin sclerosis
SSc sine scleroderma
Characterized by high titers of anti-U1RNP antibodies
Mixed connective tissue disease
Y/M: MCTD has poor response to antiinflammatory/anti-immune therapy and the prognosis is clearly worse than in patients with scleroderma
No - good response
better than in patients with classic scleroderma
Usually characterized by specific Pm-Scl autoantibodies, have typical mechanic hands, and develop early intense subcutaneous calcifications
Patients with sclerodermatous lesions who also suffer from intense myositis
Y/N: Similar to MCTD patients, patients with sclerodermatous lesions who also suffer from intense myositis respond well to an early antiinflammatory treatment
Yes
The extent and severity of skin sclerosis can be assessed by the
Modified Rodnan skin score
Y/N: Skin score at baseline correlates with disease severity and outcome in diffuse cutaneous SSc
Yes
Raynaud phenomenon appears in more than _____% of SSc patients
90
Skin involvement is a cardinal feature of SSc and usually appears first in the
Fingers and hands
Nonpitting edema of the fingers
Puffy fingers
Induration and skin thickening
Sclerodactyly
Restricted mobility of joints
Dermatogenous contractures
Reduced mouth aperture
Microstomia
Abnormal deposition of cutaneous and/or subcutaneous calcium
Calcinosis cutis
Calcinosis cutis next to joints
Thibierge-Weissenbach syndrome
Hypopigmented and hyperpigmented skin
Salt-and-pepper
Loss of hair follicles and sweat glands
Hypohidrosis/anhidrosis
Most common cause of disease-related death in SSc
Pulmonary arterial hypertension
PAH occurs in both limited and diffuse cutaneous subsets, although the most typical cases are those of
Limited SSc associated with isolated PAH
Most cases have PAH, but there are some patients with late-stage extensive _____ in SSc that develop a true secondary pulmonary hypertension
Interstitial lung fibrosis
Most common internal organ involvement in SSc
GI
Drugs that should be avoided in patients suffering from SSc
Nephrotoxic drugs
High-dose prednisolone (>7.5 mg/day)
Several studies suggest that _____ is the strongest risk factor for SSc
Positive family history
Scleroderma-like syndromes have been reported in association with
Solvents (vinyl chloride, benzene, toluene, epoxy resins)
Drugs (bleomycin, carbidopa, pentazocine, cocaine, docetaxel, metaphenylenediamine)
Coal, gold, silicon
Vasculopathy in SSc is a/an (early/late) event and is based on inappropriate vascular remodeling and repair processes
Early
Early lesions in the microcirculation because of structural damage are initially seen in
Nailfold capillaries
Vasospastic responses in RP
The first inflammatory infiltrates in lesional skin are predominantly
Cells of the monocyte lineage (T cells, macrophages, B cells, mast cells)
T cells are predominantly _____, and show a predominant _____ phenotype
CD4+
T-helper 2
Several studies suggest that B cells are able to induce ECM production through secretion of
IL-6
TGF-beta
Patients presenting only with RP should be studied for
Capillary alterations
Autoantibody status
*Predictors for the development of SSc
Number of sites assessed in the modified Rodnan score
17
Most important techniques to determine possible cardiopulmonary involvement
Pulmonary functions tests
To determine the presence of interstitial lung involvement, _____ should be used
HRCT and/or
Thoracic radiography
Gold standard to determine PAH
Right-heart catheterization
Used to detect right ventricular impairment
N-terminal brain natriuretic peptide
The best evidence available for general immunosuppression is for
Cyclophosphomide
Y/N: At present, there is no proven antifibrotic agent
Yes
Should be considered first-line therapy for RP
Calcium channel blockers
Angiotensin II receptor antagonists
Mainstay of therapy for critical digital ischemia
Help to heal digital ulcers and may prevent recurrent lesions
Parenteral prostacyclin derivatives (iloprost)
Also used especially in critical digital ischemia
Antiplatelet agents (aspirin, clopidogrel)
Therapies that are effective for PAH and digital vasculopathy
Oral dual-specificity endothelin receptor antagonist (bosentan)
Phosphodiesterase type 5 inhibitors (sildenafil, tadalafil)
Surgical treatments include ____, which can benefit single fingers with refractory ulcers
Digital microarteriolysis
May be helpful for lower-limb RP or ulceration
Lumbar sympathectomy
Key elements in the management of skin manifestations of SSc
Physical therapy and regular exercise
Ultraviolet _____ phototherapy appears to inhibit fibrotic and inflammatory processes and reduce the amount of sclerotic skin
A1
High-dose treatment with domperidone is associated with increased risk of
Cardiac arrhythmia
Distinguishes SRC from other causes of end-stage renal failure
Possibility of late recovery
Routine therapy for SRC
Angiotensin-converting enzyme inhibitors
