63 - Systemic Sclerosis Flashcards by dvojnd rivera

(Women/Men) are more frequently affected by systemic sclerosis

Women

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Age onset of systemic sclerosis

Between 30 and 50 years

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Y/N: Systemic sclerosis has the highest case-specific mortality of any of the autoimmune rheumatic diseases

Yes

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Systemic sclerosis usually starts with

Raynaud phenomenon

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Hallmark complications of systemic sclerosis

Hypertensive scleroderma renal crisis
Pulmonary arterial hypertension
Pulmonary fibrosis
GI dysmotility

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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

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Major criterion according to the American College of Rheumatology classification (1980)

Scleroderma proximal to the metacarpophalangeal or metatarsophalangeal joints

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Minor criteria according to the American College of Rheumatology classification (1980)

Sclerodactyly
Digital ulcerations and/or pitting digital scars
Bibasilar pulmonary fibrosis

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The new ACR/European League Against Rheumatism criteria considered several additional criteria such as

Abnormal nailfold capillaries
Fingertip lesions
Autoantibodies

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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

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Diffuse cutaneous SSc shows very freuqently _____ antibodies

Anti-scleroderma 70 (antitoposiomerase-I) or

Anti-RNA polymerase III

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Diffuse cutaneous SSc has a higher propensity to develop

Pulmonary fibrosis
Cardiac involvement
Scleroderma renal crisis

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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

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Limited cutaneous SSc often presents with _____ antibodies

Anticentromere

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Limited cutaneous SSc is frequently associated with

Isolated pulmonary arterial hypertension

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CREST is a _____ form of SSc

Limited

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CREST meaning

Calcinosis
RP
Esophageal dysmotility
Sclerodactyly
Telangiectasias

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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

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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

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Characterized by high titers of anti-U1RNP antibodies

Mixed connective tissue disease

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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

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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

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Y/N: Similar to MCTD patients, patients with sclerodermatous lesions who also suffer from intense myositis respond well to an early antiinflammatory treatment

Yes

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The extent and severity of skin sclerosis can be assessed by the

Modified Rodnan skin score

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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

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

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

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

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