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Stage 4: CSIM 2 > Week 12: SLE, Systemic sclerosis, Sjogren's, Antiphospholipid syndrome > Flashcards

Week 12: SLE, Systemic sclerosis, Sjogren's, Antiphospholipid syndrome Flashcards

1
Q

Pathophysiology of systemic sclerosis

A

Excess collagen deposition

T cells accumulate in skin and secrete cytokines which cause deposition of collagen

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2
Q

Components of CREST symptoms

A
  1. Calcium deposits in fingers
  2. Raynaud’s
  3. Esophageal dysmotility
  4. Sclerodactyly
  5. Telangectasia (dilated blood vessels)
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3
Q

Common symptoms in both diffuse and limited systemic sclerosis

A
  • Raynaud’s
  • Interstitial lung disease
  • Pulmonary hypertension
  • Sclerodactyly
  • Oesopheal dysmotility
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4
Q

Symptoms present in diffuse systemic sclerosis which are not present in limited systemic sclerosis

A
  • Renal (renal failure/ renal crisis)

- Cardiac (arrhythmias/ tampondade/ heart failure)

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5
Q

What are nail fold capillaroscopy findings in early and late stages of systemic sclerosis

A

Early: loss of capillaries

Late: Dilated capillaries

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6
Q

What pattern would be seen in pulmonary function tests done on a pt with systemic sclerosis

A

Restrictive pattern.

Reduced TLCO

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7
Q

Complication which may occur if giving high dose steroids to a pt with systemic sclerosis.

Clinical features of this complication.

A

RENAL CRISIS

  1. Rapid decrease in renal function
  2. Pulmonary oedema
  3. Seizures
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8
Q

Treatment and prophylaxis for a renal crisis in a pt with systemic sclerosis

A

ACEi

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9
Q

Pathophysiology of pt with Sjogren’s syndrome

A

Lymphocytes infilatrating exocrine glands

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10
Q

What systemic autoimmune diseases may be associated with Sjogren’s

A
  1. RA
  2. SLE
  3. Systemic sclerosis
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11
Q

What organ-specific autoimmune diseases may be associated with Sjogren’s

A
  1. Autoimmune thyroiditis
  2. Primary biliary cirrhosis
  3. Reno-tubular acidosis
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12
Q

Clinical features of Sjogren’s

A
  1. Dry eyes, mouth
  2. Fatigue
  3. Parotid and submandibular gland swelling/ stones
  4. Joint pain
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13
Q

Risk of Sjogren’s during pregnancy

A

Ab can cross placenta

  • cause irreversible heart block in foetus
  • cause self-limiting rash in foetus
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14
Q

Pathophysiology of SLE

A

Ab attack cell nuclear constituents and cell membranes

Due to defective clearance of apoptotic cells

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15
Q

Mucocutaneous and MSK symptoms in SLE

A
  • mouth/ nasal/ vaginal ulcers
  • dry eyes/ mouth
  • arthralgia/ arthritis + morning stiffness
  • myalgia
  • myositis
  • osteoporosis
  • tenosynovitis
  • rashes
  • photosensitivity
  • alopecia
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16
Q

What is Jaccoud’s arthropathy

A

-MCP subluxation + ulnar deviation

reversible and non-erosive cause of arthralgia

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17
Q

Renal symptoms in SLE

A
  • renal failure

- lupus nephritis (proteinuria + haematuria)

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18
Q

Neuro symptoms in SLE

A

SLE can result in any neuro manifestation

from headaches to neuropathy to stroke to depression/ anxiety/ psychosis

19
Q

Cardio symptoms in SLE

A
  • Pericarditis, pleuritic pain
  • Non-infective endocarditis
  • Hypertension
  • Vasculitis
20
Q

Haematological symptoms in SLE

A
  • Haemolytic anaemia
  • low WBC, lymphocytes, neutrophils (leukopenia)
  • low platelets (thrombocytopenia)
  • thrombosis
  • dyslipidaemia
21
Q

Why is there an increased risk of recurrent thrombosis in SLE

A

Lupus anticoagulant antibodies and anti-cardiolupin cause thrombosis

22
Q

Vascular symptoms in SLE

A

-Raynaud’s

23
Q

Respi symptoms in SLE

A
  • Pleural effusion
  • Tachypnoea
  • Fibrosing alveolitis, obliterative bronchiolitis, cough
  • Lupus pneumonitis
  • Increased risk of PE
24
Q

Eye symptoms in SLE

A
  • Dry eyes

- Conjunctivitis

25
GI symptoms in SLE
- Vomiting - Anorexia - Abdo pain - Constipation - PR bleeding
26
Pregnancy complications in SLE
- Increased risk of flare up during pregnancy - Recurrent miscarriage/ stillbirth - prematurity
27
Pregnancy complications in SLE
- Increased risk of flare up during pregnancy - Recurrent miscarriage/ stillbirth (due to increased thrombotic risk) - prematurity
28
Drugs which may cause SLE
- Antiepileptics - Sulphasalazine - Anti-TNFa drugs
29
What ethnicities have higher rates of SLE
- Asian | - Afro-Carribean
30
What CRP and ESR levels are associated with SLE
Very raised ESR with normal/ mildly elevated CRP
31
Why do patients with SLE give false positives on syphilis (VDRL) test?
There is cross reactivity between the Ab and phospholipids
32
Which CTDs are associated with cancer? | Which type of cancer?
Sjogren's: non-Hodgkin lymphoma (MALT) Dermatomyositis: solid organ malignancy
33
What blood test levels would make one concerned that Sjogren's is progressing to cancer
1. Raised ESR 2. Low complements 3. Raised immunoglobulins --> concerned about development to NHL
34
Complement levels in SLE
Low C3/ C4
35
What combination of diseases does mixed connective tissue disease consist of
1. RA 2. Myositis 3. SLE 4. Systemic sclerosis
36
Diagnostic clinical features in antiphospholipid syndrome
- recurrent DVT | - recurrent pregnancy loss
37
What type of rash is associated with antiphospholipid syndrome
Livedo rash
38
Complications of antiphospholipid syndrome
- stroke - amarosis fugax - migraines
39
Characteristic CXR of someone with sarcoidosis
Bilateral hilar lymphadenopathy
40
What is the Rose Bengal staining test for?
Shows punctate/ filmentary keratitis in eyes As part of diagnosis in Sjogren's
41
SLE requires 4/11 criteria to be diagnostic. 4 clinical signs 5 lab signs 2 rashes
SOAP BRAIN MD Clinical: SOAP 1. Serositis (pericarditis, pleurisy) 2. Oral ulcers 3. Arthritis 4. Photosensitivity Lab: BRAIN 1. Blood count disorders: pancytopenia, low complement 2. Renal involvement: proteinuria, cell casts 3. ANA 4. Immunological Ab 5. Neuro: seizures/ psychosis (with no identifiable cause) Rashes: MD 1. Malar rash 2. Discoid rash
42
What type of arthritis occurs in SLE
Non erosive | Involves 2 or more peripheral joints
43
Important lung complications of systemic sclerosis. Which is more associated with diffuse/ local
Diffuse: interstitial lung disease Local: pulmonary hypertension
44
How is APPT affected in Antiphospholipid syndrome
APPT is prolonged This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade

Stage 4: CSIM 2 (47 decks)

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