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Rheumatology > Connective tissue disease > Flashcards

Connective tissue disease Flashcards

1
Q

What is the main chemokine / cytokine involved in SLE?

A

Type 1 interferon

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2
Q

Person with negative ANA and clinical features of lupus. Can you make a lupus Dx without ANA?

A

No

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3
Q

What are teh cutaneous manifestations of SLE?

A

Acute cutaneous lupus causing a photosensitive rash the is nasolabial sparing
- Can also get facial discoid lupus that looks like a malar rash but has an area of central scaring

Subacute cutaneous lupus
- Rash that looks like psoriasis or piteraiasis

Bulus SLE

Discoid SLE

Perniosis (chillblains lupus)
Urticarial rash
Panniculitis
Allopecia

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4
Q

DO all pts with cutaneous manifestations of SLE have SLE?

A

No, some pts have isolated cutaneous manifestations
- For example only 50% of pts with subacute cut lupus have SLE.
- Only 25% of pts with discoid lupus have SLE

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5
Q

What antibody is cutaneous SLE associated with?

A

Anti-ro antibodies

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6
Q

What is the aponymous name and the features of SLE associaed small joint arthropathy?

A

Jacouds arthropathy

Deforming but non errosive small joint polyarthropathy (XR looks normal)
Nil swelling as with RA

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7
Q

In acute SLE flare, what do the ESR and CRP do? What is the exception

A

CRP is almost never elevated in SLE, even an acute flare
ESR will be elevated

The exception is serositis - CRP will be elevated

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8
Q

What is serositis in SLE?

A

Pleural effusion and pericarditis (inflammation of the thorasic linings)

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9
Q

What are the two differentials of raised CRP in SLE?

A

Infection is main one
Serositis

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10
Q

Pt comes in breathless with SLE. What is the main Dx (lung manifestation of lupus)?

A

Acute pneumonitis
Could be a PE associated with APLS (strong association with SLE)

Other less common lung manifestations inc:
- Pul HTN
- Pul haemorhage
- Shrinking lung syndroem

SLE rarely causes ILD

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11
Q

Vascular manifestations of Lupus? Cardio manifestations of lupus?

A

Vascular manifestations:
Capileritis (small vessel inflammation)
- Kayen pepper spots

Hypersensitivity vasculitis (inflam or larger vessel, often complex deposition)
- larger lesions and ulcers

Urticarial rash
- Wheel like lesions

Cardio manifestations:
- Libman sacks endocarditis (sterile endocarditis)
- Lupuis myocarditis
- Pericarditis

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12
Q

What is the most likely cardio complication of lupus?

A

Premature cardiovascular disease
(much more likely than the other more specific cardio manifestations ie pericarditis etc)

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13
Q

Haem manifestations of SLE?

A

Anaemia (often multifactorial)
- IDA, AoCD, AIHA
Leukopenia
-Most often lymphopenia, but also neutropenia
THrombocytopenia
- Large phenotypical overlap with ITP but different abs to pure ITP
MAHA (mainly TTP)
Myelofibrosis, HLH

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14
Q

What is the most important acute haem manifestation of SLE to recognize?

A

MAHA, this is an emergency

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15
Q

Neurological conditions?

A

CNS lupus
Peripheral nerve involvement:
- peripheral nerve sensory motor axonal nephropathy

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16
Q

Pt comes in with CNS manifestations, b/g SLE. What are the main 2 differentials to consider?

A

Catastrophic APLS
- If ruled out this then consider CNS lupus

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17
Q

What ab associated with CNS lupus?

A

Ribosomal P antibodies = CNS lupus

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18
Q

AUtoantibodies and specific blood findings in Lupus?

A

Raised ESR:CRP ratio

All are ANA positive (most sensitive)
Anti Smith (most specific)
Anti dsDNA

Anti SSA (anti ro), Anit SSB (la) can be positive, mostly associated weith sjogrens syndrome

Anti U1RNP - most often associated with MCTD but can be positive in Lupus

Anti ribosomal P - mostly with renal and CNS lupus

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19
Q

COngenital heart block is associated with which connective tissue disease?

A

SLE

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20
Q
A
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21
Q

What autoantibody associated with development of congenital heart block?

A

Anti SSA (anti ro52)
- Also anti SSB (anti la)

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22
Q

What is a neonatal cuteneous complications of lupus?

A

… neonatal cutaneous lupus lol
- due to autoantibodies that cross the placental and cause manifestations on the baby. Rarely severe disease, usually resolves

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23
Q

Rx of Lupus?

A

Non pharm:
- UV protection
- Smoking ceasation

Pharm:
- Hydroxychloroquine (backbone)
- Steroids (acute flares)
- Mycophenylate (renal lupus)
- Cyclosporin, tac (renal lupus)
- MTX, AZA, Leflunamide
- Cyclophosphamide

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24
Q

What drug should all lupus pts be on (similar to MTX in RA)?

A

Hydroxychloroquine

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25
Q

What is the main side effect of long term hydroxychloroquine? what other similar drugs can cause this same manifestation?

Who is most at risk of this complication? How is it monitored?

A

Retinopathy
- 304% for pts on HCQ >20 years

Mainly recognized in use of chloroquine for malaria. HCQ is a similar but different medication

Most at risk - elederly, renal impariment

Monitoring - HCQ >5 years needs annual eye checks to monitor. This is because it is slowly progressive and reversible in early stages

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26
Q

What is Belimumab? what does it target?

A

This is a mab to BLySS/BAFF pathway in SLE
- Not PBS approved but TGA approved

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27
Q

What is anifrolumab?

A

Anifrolumab is a mab directed at the subunit of the INF alpha receptor
- Not PBS approved but TGA approved for SLE

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28
Q

Pt with SLE, presents with GN and PE. APLS negative. WHy do they have a PE?

A

GN associated with hypercoagulability, especcially nephrotic syndromes (Lupus nephritis class V (membranous nephritis)

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29
Q

What is the classic cutaneous sign of APLS?

A

Livedo reticularis

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30
Q

What are the three tests in APLS?

A

Anti beta 2 glycoprotein
Anti cardiolipin

Check IgG and IgM of both the above, IgG is more relevant, therefore if have high IgG titre then this will be worse

Lupus anticoagulent (not an actual antibody, this is a functional test)
- contributes to thrombosis risk in vivo, but is an anticoagulent in vitro

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31
Q

What antibody / test in APLS is most associated with thrombosis?

A

Lupus anticoagulant

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32
Q

Young person with prolongued APTT? what is an important differential to consider that could solely manifest as prologue APTT on routine bloods?

A

APLS

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33
Q

How is APLS treated?

A

Primary prevention
- ? low dose aspirin or HCQ (somewhat contentious)

Secondary prevention (ie they have already had a clot / preg event)
- Warfarin INR 2-3 Indefinitley
- NOACs dont work

Management in pregnancy
- Prior thrombosis: therapeutic LMWH and aspirin
- Prior preg loss: proph LMWH and aspirin

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34
Q

Skin features of dermatomyositis?

A

Gotrons papules, and gotrons sign (rash of elbows or knees)
Shawl sign

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35
Q

What are the essential features of antisynthetase syndrome?

A

This is a specific subtype of myositis (usually dermato or polymyositis)

Key features:
- Myositis (ie elevated CK, may have clinica signs)
- ILD
- Mechanics hands - hyperkeratiosis and fisuring of the fingers and thumb
- Raynauds - not common with other forms of myositis
- Inflammatory polyarthritis
- Oesophageal dysmotility - dysphagia

36
Q

What is the main auto-antibody in antisynthetase syndrome?

A

Anti Jo1

37
Q

Pt with myositis has anti-HMGCR? what condition?

A

Immune mediated necrotising myositis, probably statin associated but not always

38
Q

What HLA is IMNM associated with?

A

HLADRB1 11*01

39
Q

Pt with dermatomyositis has MDA5+. What are they most at risk of developing?

A

ILD

40
Q

What are the features of clinically amyopathic dermatomyositis (MDA5+ dermatomyositis)?

A

Often nil muscle involvement clinically (clinically amyopathic)
- Associated with rapidly progressive ILD and high mortality
- therefore need aggressive immunosupression

  • Cutaneous ulcerations
  • Inverse gottrens papules (palmer aspect rather than dorsal surface
  • Pneumomediastinum
41
Q

What are the three areas of pathophysiology in systemic sclerosis?

A

Vascular damage
Autoimmunity
Tissue fibrosis

42
Q

Pt presents with PHTN and finger tip ulcers. What condition?

A

scleroderma
- very important cause of PHTN, often the first presentation of the disease

43
Q

Autoantibodies in SSc?

A

Anti centromere
- limited cutaneous systemic sclerosis, therefore main complicaiton is PHTN

Anti-topoisomerase 1(anti SCL70)
- associated with difuse SSc. therefore main complication is associated ILD

Anti RNA polymerase III
- severe renal involvement

44
Q

What is the pattern of skin involvement in limited cutaneous SSc?

A

Distal skin thickening (below elbows)
- therefore if pt has diffuse skin thickening with proximal arm or face/trunk affected then this is diffuse

45
Q

What is a key feature that almost all pts with SSc have, often before they receive the diagnosis?

A

Raynauds
- would be very unusual to get SSc without raynauds. IN limited disease, often have long Hx raynauds before present, as opposed to diffuse disease where the Hx of raynauds is typically much shorter

46
Q

Explain CREST?

A

Calcinosis
Raynauds
Esophageal inv
Sclerodactyly
Telangioectasia

47
Q

What is systemic sclerosis sine scleroderma?

A

THis is systemic sclerosis with nil skin involvment
- Ie pt has ILD due to SSc, but nil skin involvement

48
Q

Pts with SSc can get cardiac involvment. What is an important manifestation of cardiac involvment?

A

Conduction disease due to cardiac fibrosis

49
Q

What is the scleroderma renal crisis presentation?

A

This manifests as an accelerated hypertension type phenotype
HTN may be asymptomatic of symptomatic. Symptoms/signs including:
- Headache, visual change
- Papiloedema
- Cardiac failure

50
Q

Pt with systemic sclerosis has HP 110/80. Comes back 1 month later for routine check, now BP 150/95. What should you be concerned about

A

Developing scleroderma renal crisis
Often will develop accelerated HTN, then the renal crisis

51
Q

What medication should be avoided in SRC?

A

Corticosteroids

52
Q

What is the main treatment for SRC?

A

ACEI/ARB

53
Q

When I move my ankle creaks. Exam shows tendon crepitus. What condition

A

Systemic SSc, this is tendon involvement (tendon rub)
Indicator of bad prognosis

54
Q

What are the GI abn in SSc?

A

CREST is obvs associated with oesophageal involvment
however any part of the GIT can be affected

Other GIT manifestations inc:
- GAVE (watermelon stomach on G scope), delayed gastric emptying
- Small bowel overgrowth
- Faceal loading and secondary incont, or bowel obstruction

55
Q

What are the phases of raynauds?

A

White, blue/purple, red

56
Q

What distinguishes primary raynauds to secondary?

A

Secondary
- painful raynauds (most raynauds in uncomfortable but not painful)
- Other manifestation ie skin etc

57
Q

Adult 40yo with new raynauds. Should you be concerned?

A

Every cause of new onset raynauds in adulthood has an important cause, needs to be investigated
- Primary raynauds begins in teenage years, secondary raynauds usually onsets in adulthood

58
Q

Abnormal nail fold capilioscopy is usually assoicated with which connective tissue disease?

A

systemic sclerosis

59
Q

What is a complication of raynauds? how does it manifest and time course?

A

Digital ulcers
- Extremely tender, last for several weeks to months
- Can be very debilitating

60
Q

What is the main risk during preganncy for systemic sclerosis?

A

Increased risk of Preeclapsia and ecclampsia

61
Q

Typical treatment regime for SSc ILD?

A

Nintedanib (antifibrotic) and mycophenylate

62
Q

What is mixed connective tissue disease?

A

Currently this is a distinct entity however the phenotypes is that of a SLE and scleroderma overlap

63
Q

Pt with puff hands. What disease?

A

MCTD

64
Q

What is the commonest cause of death (therefore the most important complication) of MCTD?

A

Pul HTN

65
Q

Characteristic lab findings in MCTD? How to tell between lupus and MCTD with antibodies?

A

Positive ANA, speckled pattern
Anti U1RNP (high titre), anti SSA (anti ro) often positive

There is often overlap between antibodies in SLE and MCTD. However if pt has pos Anit-sm, anti-dsDNA, or anti ribosomal P antibodies then it is lupus

Almost all MCTD have pos RF, but negative anti CCP. If pt has positive anti CCP then this is RA

66
Q

Sjogrens syndrome can be thought of as a disease of the … ?

A

Exocrine organs

67
Q

What are the main symptoms of SS? what is the pathological cause of these symptoms?

A

Sicca symptoms (xerostomia, xeropthalmous of the eye, dry vagina)
- Due to lymphocytic infiltration of the exocrine glands
Gladular enlargment
Circulating antibodies

68
Q

What are some DDx of parotid enlargment?

A

Infections
- Viral: mumps, EBV, HCV, HIV
- Bacterial: acute bacterial parotitis, TB
Autoimmiune
- Sjogrens syndrome, GPA
Inflamatory:
- IgG4 disease
Metabolic:
- Diabetes, Bulimia, alcoholism
Cancer:
- Lymphoma, leukameia
Granulomatous
- sarcoidosis

69
Q

Palpable purpura. Dx?

A

Sjogrens disease

70
Q

Pt with Sjogrens syndrome has thin walled cystic leions on HRCT. What is the Dx?

A

Lymphocytic interstitial pneumonia

71
Q

Pt has lymphocytic intersitial pneumonia, what primary condition do they most likely have?

A

Sjogrens syndrome

72
Q

Pt with NAGMA and Sjogrens, what is teh Dx?

A

Type 1 RTA

73
Q

What is teh main renal manifestation of Sjogrens syndrome?

A

TYpe 1 RTA

74
Q

Pt has mixed cryoglobulinaemia, which connective tissue disease do they have? What would be the most likely infection associated with this?

A

Sjogrens syndrome
Hep C virus

75
Q

What is a characteristic but uncommon neurological manifestation of Sjogrens syndrome? How does it manifest?

A

Sensory ganglionopathy that manifests as pseudoathetosis due to lack of proprioception in the affected limb

76
Q

What cancer is Sjogrens syndrome most associated with?

A

Lymphoma (NHL, most common is MALT)

77
Q

Autoantibodies in Sjogrens syndrome?

A

Most common:
- anti SSA (anti Ro60), Anti SSB (anti La)

Often have RF positive, CCP will be negative
Often have polyclonal hypergammaglobulinaemia -30%

Sometimes have cryoglobulins 15%

78
Q

What is the antibody associated with neuromyelitis optica?

A

AQP4 IgG ab positive

79
Q

What are the cardinal features of Behcets disease?

A

Relapsing oral and genital ulcers with bilateral posterior or panuveitis (hypopyona in anterior uveitis)
- often in a pt from the silk road demographic

Other features:
- pustulopapular rash (mucosal and non mucosal)
- non errosive mono or oligo arthritis
- Pathergy (abnormal reaction that occurs after tissue damage ie venepuncture)
- Pan vasculitis

80
Q

What HLA in Behcets disease?

A

HLA B 51

81
Q

What pathological description is characteristic of sarcoidosis?

A

Non caseating greanulomatous inflamation

82
Q

Lupus pernio. What condition?

A

Sarcoidosis?

83
Q

What are some key rheum features of sarcoidosis? What is lofgrens syundrome?

A

Lupus pernio
Errythema nodosum
Isolated ankle arthritis

Lofgrens syndrome is a clinical triad of sarcoidosis:
- Fever
- EN
- bilateral hilar Lymphadenopathy

84
Q

Pt has painful ears. What condition?

A

Relapsing polychondritis

85
Q

What is teh main malignant associated of relapsing polychondritis?

A

Myelodysplasia

86
Q

What is teh pathopneumonic histo for IgG4 disease?

A

Lymphocytic infiltrate
Storiform fibrosis

87
Q

What are the most common / important manifestation of IgG4 disease?

A
  • Retroperitoneal fibrosis
  • Periaortitis, periarteritis (manifesting as vascular complications)
  • renal ionvolment

Rheumatology (4 decks)

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