Exam 9 Nmsk77 - Connective Tissue Diseases

Front 1

What connective tissue disease exerts an increased risk in lymphoma?

Back 1

Sjogren’s Syndrome

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Connective tissues diseases:

-Perivascular ... deposition=... Vascular Diseases
-Autoimmune diseases
-Exact cause remains ...
-Different diseases associated with specific ...

Back 2

collagen
Collagen
obscure
autoantibodies

Front 3

Connective tissue diseases are those syndromes manifest as ... involvement in which the pathogenesis is heavily dependent on self-directed ... mechanisms

Examples
SLE, MCTD, SS, PSS, polymyositis

Back 3

multiple organ system
immune

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disease – autoantibody:

... – anti-dsDNA, Anti-SM
... – RF, Anti-RA33
... – Anti-Ro(SS-A), Anti-La(SS-B)
... – Anti-Scl-70, Anti-centromere
... – Anti-Jo-1
... – Anti-U1-RNP
... – c-ANCA

antibodies sometimes are overlapping

Back 4

Systemic Lupus Erythematosus (SLE)
Rheumatoid Arthritis (RA)
Sjogrens Syndrome
Systemic Sclerosis
Polymyositis/Dermatomyositis
Mixed Connective Tissue Disease
Wegener’s Granulomatosus

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These diseases are grouped together because they are ..., that is they affect the body as a whole through involvement of multiple organ systems, they require ... activation and in general they share epidemiologic predispositions and a similar response to ....

Back 5

systemic
immune
immunosuppression

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...:

autoimmune multisystem disease
prevalence 1 in 2,000
9 to 1; female to male (1 in 700)
peak age 15-25
immune complex deposition
photosensitive skin eruptions, serositis, pneumonitis, myocarditis, nephritis, CNS involvement

Back 6

Systemic Lupus Erythematosus

Front 7

To be diagnosed as having lupus (SLE), you have to have a positive fluorescent ...

Back 7

antinuclear antibody (ANA)

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SLE criteria definitions:

... – fixed erythema, flat or raised, sparing the nasolabial folds
... – raised patches, adherent keratotic scaling, perifollicular plugging. Older lesions may cause scarring
... – skin rash from sunlight
... – usually painless
... – NONEROSIVE, inflammatory in 2 or more peripheral joints
... – results in pleuritis or pericarditis

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malar rash
discoid rash
photosensitivity
oral or nasopharangeal ulcers
arthritis
serositis

Front 9

ANA ... “the lupus test”. Many more “normal” people are positive for it than are people with lupus. However, its absence speaks strongly ... a patient having SLE. It is still unclear as to whether ANAs are part of the pathology or an epiphenomenon caused as a consequence of the true pathology.

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are not
against

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SLE shows a deformity of ... arthritis

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non-erosive

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There is no 100% definite trigger, but the only well documented trigger for LE is ...

Back 11

UV light

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... or ... consequences of SLE = Nephritis, thrombocytopenia, cerebritis, thromboembolism, hemolytic anemia, seizures, lupus pneumonitis, AVN, psychosis

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serious, life-threatening

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... – close cousin of SLE

-Chronic disorder characterized by immune-mediated destruction of exocrine glands
-Primary vs Secondary:
-Primary is diagnosis of exclusion
-Secondary refers to the sicca complex accompanying any of the connective tissue diseases (xerophthalmia, keratoconjuntivitis, xerostomia with/without salivary gland enlargement)

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Sjogren Syndrome

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Sjogren Syndrome:

-1% of the population and in 10-15% of RA patients
-9:1 female:male preponderance
-Age of onset 40-60 years
-Associated with a 33-44 times increased risk of ....
-dry eyes and dry mouth

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lymphoma

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... – more severe as compared to SLE or Sjogrens syndrome

-Also known as systemic sclerosis
-Sclerotic skin changes often accompanied by multisystem disease.
-Progressive fibrosis from increased collagen deposition in intersitium and intima of small arteries and connective tissues
-May be benign cutaneous involvement or aggressive systemic disease.

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Scleroderma

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Scleroderma diagnostic criteria:

One major criterion: scleromatous skin changes proximal to the ... joints

Two of three minor criteria: sclerodactyly, digital pitting scars, bi-basilar pulmonary fibrosis on CXR

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metacarpal-phalangeal (MCP)

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Scleroderma:

presentation
-... phenomenon (intense vasospasm in hands and feet, especially when cold)
-edema fingers and hands
-skin ...

visceral manifestations
-GI tract, lung, heart, kidneys (scleroderma renal crisis), thyroid

arthralgias and muscle weakness often

contractures and extra calcification (in hand)

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Raynaud’s
thickening

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Scleroderma: head and neck manifestations

What is the most common initial complaint?

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dysphagia – due to fibrosis of esophagus

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Treatment of scleroderma:

Focus on ...
-Antibiotics for bowel motility
-... for Raynaud's

Treat responsive syndromes
-PAH with bosentan, flolan, Rovatio
Renal crisis with ...

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symptoms
Gloves
ACE inhibitors

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Summary

-CTD are ...-system diseases
-... mechanisms are key to their pathogenesis and therapy
-Diagnosis are clinically based using criteria
-Treatment is non-specific-aimed instead at specific ...
-A move toward prevention of key complications like CAD and renal crisis is in progress

Back 20

multi
Immune
manifestations