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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
Q

GI symptoms in SLE

A
  • Vomiting
  • Anorexia
  • Abdo pain
  • Constipation
  • PR bleeding
26
Q

Pregnancy complications in SLE

A
  • Increased risk of flare up during pregnancy
  • Recurrent miscarriage/ stillbirth
  • prematurity
27
Q

Pregnancy complications in SLE

A
  • Increased risk of flare up during pregnancy
  • Recurrent miscarriage/ stillbirth (due to increased thrombotic risk)
  • prematurity
28
Q

Drugs which may cause SLE

A
  • Antiepileptics
  • Sulphasalazine
  • Anti-TNFa drugs
29
Q

What ethnicities have higher rates of SLE

A
  • Asian

- Afro-Carribean

30
Q

What CRP and ESR levels are associated with SLE

A

Very raised ESR with normal/ mildly elevated CRP

31
Q

Why do patients with SLE give false positives on syphilis (VDRL) test?

A

There is cross reactivity between the Ab and phospholipids

32
Q

Which CTDs are associated with cancer?

Which type of cancer?

A

Sjogren’s: non-Hodgkin lymphoma (MALT)

Dermatomyositis: solid organ malignancy

33
Q

What blood test levels would make one concerned that Sjogren’s is progressing to cancer

A
  1. Raised ESR
  2. Low complements
  3. Raised immunoglobulins

–> concerned about development to NHL

34
Q

Complement levels in SLE

A

Low C3/ C4

35
Q

What combination of diseases does mixed connective tissue disease consist of

A
  1. RA
  2. Myositis
  3. SLE
  4. Systemic sclerosis
36
Q

Diagnostic clinical features in antiphospholipid syndrome

A
  • recurrent DVT

- recurrent pregnancy loss

37
Q

What type of rash is associated with antiphospholipid syndrome

A

Livedo rash

38
Q

Complications of antiphospholipid syndrome

A
  • stroke
  • amarosis fugax
  • migraines
39
Q

Characteristic CXR of someone with sarcoidosis

A

Bilateral hilar lymphadenopathy

40
Q

What is the Rose Bengal staining test for?

A

Shows punctate/ filmentary keratitis in eyes

As part of diagnosis in Sjogren’s

41
Q

SLE requires 4/11 criteria to be diagnostic.

4 clinical signs
5 lab signs
2 rashes

A

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
Q

What type of arthritis occurs in SLE

A

Non erosive

Involves 2 or more peripheral joints

43
Q

Important lung complications of systemic sclerosis.

Which is more associated with diffuse/ local

A

Diffuse: interstitial lung disease

Local: pulmonary hypertension

44
Q

How is APPT affected in Antiphospholipid syndrome

A

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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