Connective Tissue Disorders Flashcards by Meley Gdey

list key connective tissue disorders

systemic lupus erythematous
Sjorgen's syndrome
autoimmune inflammatory muscle disease
systemic sclerosis (scleroderma)
overlap syndromes

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what is rheumatoid arthritis?

chronic joint inflammation that can result in joint damage

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site of inflammation of rheumatoid arthritis

synovium

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rheumatoid arthritis is associated with?

autoantibodies

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what is ankylosing spondylitis?

chronic spinal inflammation that can result in spinal fusion and deformity

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site of inflammation of ankylosing spondylitis

includes the enthesis

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is ankylosing spondylitis associated with autoantibodies?

‘seronegative’

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list seronegative spondyloarthropathies

ankylosing spondylitis
reactive arthritis (Reiter’s syndrome)
Psoriatic arthritis
arthritis associated with GI inflammation (enteropathic synovitis)

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what is systemic lupus erythematosus?

chronic tissue inflammation in the presence of antibodies directed against self antigens

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site of inflammation of SLE?

multi site

particularly the joints, skin, kidney

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is systemic lupus erythematosus associated with autoantibodies?

yes
anti nuclear antibodies
anti-double stranded DNA antibodies
anti-phospholipid antibodies

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list connective tissue diseases

SLE
Sjogren's syndrome
autoimmune inflammatory muscle disease
systemic sclerosis
overlap syndrome

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what phenomenon is common in connective tissue disorders?

Raynaud’s phenomenon

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what is Raynaud’s phenomenon?

intermittent vasospasm of digits on exposure to cold

colour change: blanching of digits, cyanosis, reactive hyperaemia

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typical demographic for SLE

female

15-45

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clinical manifestations of SLE include

malar rash
photosensitive rash
mouth ulcers
hair loss
Raynaud's phenomenon
arthralgia/arthritis
serositis e.g. pericarditis, pleuritis
renal disease e.g. glomerulonephritis
cerebral disease e.g. psychosis

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current paradigm for pathogenesis of systemic lupus erythematosus

apoptosis > translocation of nuclear antigens to membrane surface > impaired clearance of apoptotic cells > enhanced presentation of nuclear antigens to immune cell > B cell autoimmunity > tissue damage by antibody effector mechanisms e.g. complement activation and Fc receptor engagement

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what autoantibodies are involved in systemic vasculitis?

antinuclear cytoplasmic antibodies

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antinuclear antibody screening includes?

Anti-Ro
Anti-La
Anti-centromere
Anti-Sm
Anti-RNP
Anti-ds-DNA antibodies 
Anti-Scl-70

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cytoplasmic autoantibodies include?

Anti-tRNA synthetase antibodies

Anti-ribosomal P antibodies

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which autoantibodies are specific for SLE?

anti dsDNA

anti Sm

antiphospholipid antibodies are associated with the risk of what in SLE?

arterial and venous thrombosis

serum level of anti-dsDNA antibody correlates with?

disease activity

treatment for SLE aims for?

remission or low disease activity and prevention of flares

drug treatments for SLE patients

hydroxychloroquine recommended maintenance treatment glucocorticoids minimised (can be helped by methotrexate, azathioprine) in persistently active/severe disease use cyclophosphamide and B cell targeted therapies e.g. rituximab/belimumab

patients with SLE should be assessed for?

antiphospholipid antibody status infectious and cardiovascular diseases risk profile pregnancy planning

disease activity in SLE

``` low complement C3/C4 high anti-dsDNA antibodies low platelet count low albumin low Hb, lymphocyte high reticulocyte ```

what is sjogren's syndrome?

chronic autoimmune disease affecting salivary glands and tear glands, autoimmune exocrinopathy

exocrine gland pathology results in?

``` dry eyes (xerophthalmia) dry mouth (xerostomia) parotid gland enlargement ```

Commonest extra-glandular manifestations in Sjogren's syndrome are?

non-erosive arthritis | Raynaud’s phenomenon

salivary gland biopsy of patient with Sjogren's syndrome will show?

lymphocytic infiltration predominantly CD4 helper T cells and to lesser extent B lymphocytes

what is Schirmer's test?

a test to assess tear production. Filter paper is placed under lower eyelid and extent of wetness measured after 5 minutes

abnormal result of Schirmer's test is?

<5mm after 5 minutes

Sjogren's syndrome typical demographic

females | 35-50

inflammatory muscle disease

Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash

skin changes in dermatomyositis

Lilac-coloured (heliotrope) rash on eyelids, malar region and naso-labial folds Red or purple flat or raised lesions on knuckles (Gottron’s papules) Subcutaneous calcinosis Mechanic’s hands (fissuring and cracking of skin over finger pads)

inflammatory muscle disease is associated with?

malignancy | pulmonary fibrosis

inflammatory muscle disease will have what abnormal test results?

elevated CPK abnormal electromyography abnormal muscle biopsy (polymyositis = CD8 T cells; dermatomyositis = CD4 T cells in addition to B cells)

what is systemic sclerosis?

group of diseases causing hardening and tightening of skin and connective tissues.

manifestations of systemic sclerosis

dermal fibrosis | cutaneous calcinosis telangiectasia

skin changes in systemic sclerosis may be ________ or ______

diffuse | limited

diffuse systemic sclerosis

Fibrotic skin proximal to elbows or knees (excluding face and neck) Anti-topoisomerase-1 (anti-Scl-70) antibodies Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement Short history of Raynaud’s phenomenon

limited systemic sclerosis

Fibrotic skin hands, forearms, feet, neck and face Anti-centromere antibodies Pulmonary hypertension Long history of Raynaud’s phenomenon

CREST describes a sub-type of limited systemic sclerosis, it stands for?

``` Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangietasia ```

what is overlap syndrome?

features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease

what is undifferentiated connective tissue disease?

When incomplete features of a connective tissue disease are present

what is mixed connective tissue disease?

In one instance a group of patients with features of seen in SLE, scleroderma, rheumatoid arthritis, and polymyositis were identified by the presence of an autoantibody: Anti-U1-RNP antibody

key autoantibodies in diffuse systemic sclerosis

anti-Scl-70 antibody

key autoantibodies in limited systemic sclerosis

anti-centromere antibodies

key autoantibodies in dermato-/polymyositis

anti-tRNA transferase antibodies

key autoantibodies in Sjogren's syndrome

no unique antibodies but typically see antinuclear antibodies (anti-Ro and anti-La) rheumatoid factor

key autoantibodies in mixed connective tissue disorders

anti-U1-RNP antibodies