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Y2 Rheumatology > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

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1
Q

What are Connective Tissue Diseases?

A 
Multisystem disorders that can cause organ pathology
SLE
Sjogrens
Systemic Sclerosis
Mixed Connective Tissue Disease
Anti-phospholipid Syndrome
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2
Q

What is the proposed pathogenesis of SLE?

A

Defect in apoptosis –> increased cell death
Defective clearance of apoptotic cell debris –> persistence of antigen & immune complex production
Circulating immune complexes with antigens in various tissues

in small BVs: complement activation & inflammation
BMs of skin & kidneys

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3
Q

Environmental & genetic basis of SLE

A

UK : &S: Black&raquo_space; white (however disease rarely reported in Africa)
40% concordance in MZ

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4
Q

F:M of SLE

A

F:M is 11:1 in childbearing years

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5
Q

onset age of SLE

A

after puberty - 20-30s

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6
Q

% of SLE cases in women

A

90%

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7
Q

Constitutional signs & symptoms of SLE

A

fever
fatigue
weight loss

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8
Q

Other typical signs & symptoms of SLE (there’s way too many)

A 
arthralgia, myaliga, AVN maybe
malar rash, photosensitivity
discoid lupus, Raynaud's
lupus nephritis
lung
haematological
neuropsychiatric
cardiac
GI
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9
Q

Investigation of SLE

A

no one diagnostic test
FBC may show: anaemia, leucopenia, thrombocytopenia
urinalysis: look for glomerulonephritis
imaging: CT, MRI, Echo (organ involvement)
serology

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10
Q

Serology for SLE

A

ANA: high sensitivity, low specificity
Anti-dsDNA: varies with disease activity (so may not be super sensitive), high specificity
Anti-Sm: low sensitivity, high specificity
C3/4: sensitivity low when disease is active

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11
Q

Specific disease markers for SLE

A

Anti-dsDNA, Anti-Sm

ANA not specific

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12
Q

Sensitive disease markers for SLE

A

ANA very sensitive (not specific though)
Anti-dsDNA varies with disease activity
(Anti-Sm low sensitivity)

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13
Q

Which marker is used for disease activity in SLE

A

Anti-dsDNA

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14
Q

Which marker is used for disease activity in SLE

A

Anti-dsDNA

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15
Q

SLICC classification criteria for SLE

A

4 or more criteria with at least 1 clinical & 1 laboratory
or biopsy-proven lupus nephritis with positive ANA / Anti-DNA

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16
Q

Clinical Criteria for SLE (11)

A 
Acute Cutaneous Lupus
Chronic Cutaneous Lupus
Oral / Nasal Ulcers
Non-scarring alopecia
Arthritis
Serositis
Renal
Neurologic
Haemolytic anaemia
Leukopenia
Thrombocytopenia
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17
Q

Immunologic Criteria for SLE

A 
ANA
Anti-DNA
Anti-Sm
Antipohspholipid Ab
Low complement (C3,4)
Direct Coomb's test
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18
Q

Treatment of SLE of there is skin disease & arthralgia

A

hydroxychloroquine
topical steroids
NSAIDs

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19
Q

Treatment of SLE if there is inflammatory arthritis/ evidence of organ involvement (pericardial disease, ILD)

A

immunosuppression: azathioprine /myocephanolate mofetil

Corticosteroids at moderate doses for short periods

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20
Q

Treatment of SLE if there is severe organ disease (lupus nephritis / CNS lupus)

A

IV steroids, cyclophosphamide

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21
Q

Treatment of unresponsive SLE

A

IV immunoglobulin, rituximab

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22
Q

Monitoring of SLE

A

according to symptoms
check regularly: Anti-dsDNA, C3/4
urinalyiss- check for glomerulonephritis
evaluate & manage CV risk factors - BP, cholesterol

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23
Q

What is the pathogenesis of Sjogrens Syndrome?

Study These Flashcards
A

Autoimmune - lymphocytic infiltrates in exocrine organs

24
Q

Aetiology of Sjogrens Syndrome:

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A

Primary

Secondary to other AI - RA, SLE

25
Symptoms of Sjogrens Syndrome
sicca symptoms: dry eye & mouth other symptoms: arthralgia, fatigue, vaginal dryness, parotid gland swelling may also occur: peripheral neuropathy & ILD, increased risk of lymphoma
26
Diagnosis of Sjogrens Syndrome
4 of the following: occular symptoms: -dry eyes for > 3 months -foreign-body sensation -use of tear substitutes > 3x/day oral symptoms: -dry mouth -recurrently swollen salivary glands -frequent use of liquids to aid swallowing ocular signs -Schimer test without anaesthesia (<5mm in 5min) -positive vital dye stainint result oral signs -abnormal salivary scintigraphy findings -abnormal parotid gland scintigraphy findings -abnormal sialometry findings -unstimulated salivary flow <1.5 mL in 15 min positive minor salivary gland biopsy findings positive anti-SSA/anti-SSB (Ro or LA)
27
Serology in Sjogrens Syndrome
Anti-SSA/Anti-SSB (Ro / LA)
28
Oral Symptoms & Signs of Sjogrens Syndrome
``` Symptoms: -dry mouth -recurrent swollen salivary glands -frequent use of liquids to aid swallowing Signs: -abnormal salivary scintigraphy findings -abnormal parotid scintigraphy findings -abnormal sialometry findings -unstimulated salivary flow <1.5 mL in 15 min ```
29
Oral Symptoms & Signs of Sjogrens Syndrome
``` Symptoms: -dry mouth -recurrent swollen salivary glands -frequent use of liquids to aid swallowing Signs: -abnormal salivary scintigraphy findings -abnormal parotid scintigraphy findings -abnormal sialometry findings -unstimulated salivary flow <1.5 mL in 15 min ```
30
What is the Pathogenesis of Systemic Sclerosis
Excessive Collagen Deposition leading to skin & internal organ changes Vasomotor disturbances (Raynaud's) Fibrosis --> Atrophy of skin & subcutaenous tissue 3 phases of cutaneous symptoms: odematous, indurative, atrophic
31
3 phases of cutaneous symotoms of SSC
odematous indurative atrophic
32
What leads to death in SSC?
renal & lung changes | pulmonary hypertension leads to 12% of SSC-related deaths
33
Signs & Symptoms of SSC
``` Raynaud's phenomenon Thickened & Tight skin -skin of face: pinching of the skin of nose (beaking) -tightening of the skin around the mouth Telangiectasia Calcinosis - may be seen in digits ```
34
Major & Minor Features of SSC
``` Major: -centrally located skin sclerosis -arms, face, neck Minor: -sclerodactyly & atrophy of fingertips -bilateral lung fibrosis ```
35
Organ involvement in SSC
lung: pulmonary HTN, pulmonary fibrosis accelerated HTN --> renal crisis gut: dysphagia, malabsorption, bacterial overgrowth of small bowel MSK: inflammatory arthritis & myositis
36
Limited SSC other name
CREST
37
Limited SSC disease progression
skin involved confined to face, hands, forearms & feet | organ involvement occurs later
38
Diffuse SSC disease progression
skin changes develop more rapidly, can involve the trunk | early significant organ involvement
39
Limited SSC Ab
Anti- Centromere
40
Diffuse SSC Ab
Anti-Scl-70
41
Investigations of SSC
``` Immunology Organ screening regularly: -pulmonary function test, -echocardiogram, -monitor renal function ```
42
Diagnosis of SSC
1 major & 2 minor features
43
Major features of SSC
centrally located skin sclerosis | arms, face, neck
44
Minor features of SSC
sclerodactyly and atrophy of the fingertips | bilateral lung fibrosis
45
Treatment of SSC
Raynauds/digital ulcers: CCBs (Iloprost, Bosentan) Renal involvement: ACEi GI involvement: PPI for reflux ILD: immunosuppression - cyclophosphamide
46
Features & Symptoms of MCTD
``` Raynauds Arthralgia/Arthritis Myositis Sclerodactyly Pulmonary HTN ILD ```
47
Serology for MCTD
anti-RNP Ab
48
Monitoring of MCTD
Regular echocardiograms - risk of pulmonary htN | Pulmonary function test - screen for ILD
49
Treatment of MCTD
Depends on presentation Raynauds: CCBs (Iloprost, Bosentan) Muscle/lung disease: immunosuppression
50
What is the pathology behind Anti-Phospholipid Syndrome?
Recurrent venous/arterial thrombosis and/or fetal loss
51
Associated diseases to Anti-Phospholipid Syndrome
SLE or other AI disorders
52
Signs & Symptoms of Anti-Phospholipid Syndrome
- Increased frequency of stroke/MI - Recurrent pulmonary emboli/thrombosis - pulmonary HTN - Catastrophic ACS (CAPS): multiorgan infarction over a period of days-weeks - Late spontaneous fetal loss (2nd or 3rd trimester) - Recurrent early fetal loss (10 weeks) also possible - Migraine - Livedo reticularis
53
In Anti-Phospholipid Syndrome, what is stroke due to?
In situ thrombosis | Embolization origination from the valvular lesions of Libman-Sacks (Sterile) Endocarditis
54
What is a common cutaneous finding in APS?
Livedo Reticularis
55
Investigations of APS
Thrombocytopenia Prolongation of APTT Immunology
56
Serology of APS
Lupus ANticoagulant Anti-cardiolipin Anti-beta 2 glycoprotein
57
Treatment of APS
episode of thrombosis: Anti-coagulation recurrent pregnancy loss: LMWH during pregnancy (Warfarin would be teratrogenic) Positive Ab without thrombosis event: don't require anticoagulation

Y2 Rheumatology (7 decks)

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