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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
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?
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
26
What is Belimumab? what does it target?
This is a mab to BLySS/BAFF pathway in SLE - Not PBS approved but TGA approved
27
What is anifrolumab?
Anifrolumab is a mab directed at the subunit of the INF alpha receptor - Not PBS approved but TGA approved for SLE
28
Pt with SLE, presents with GN and PE. APLS negative. WHy do they have a PE?
GN associated with hypercoagulability, especcially nephrotic syndromes (Lupus nephritis class V (membranous nephritis)
29
What is the classic cutaneous sign of APLS?
Livedo reticularis
30
What are the three tests in APLS?
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
31
What antibody / test in APLS is most associated with thrombosis?
Lupus anticoagulant
32
Young person with prolongued APTT? what is an important differential to consider that could solely manifest as prologue APTT on routine bloods?
APLS
33
How is APLS treated?
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
34
Skin features of dermatomyositis?
Gotrons papules, and gotrons sign (rash of elbows or knees) Shawl sign
35
What are the essential features of antisynthetase syndrome?
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
What is the main auto-antibody in antisynthetase syndrome?
Anti Jo1
37
Pt with myositis has anti-HMGCR? what condition?
Immune mediated necrotising myositis, probably statin associated but not always
38
What HLA is IMNM associated with?
HLADRB1 11*01
39
Pt with dermatomyositis has MDA5+. What are they most at risk of developing?
ILD
40
What are the features of clinically amyopathic dermatomyositis (MDA5+ dermatomyositis)?
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
What are the three areas of pathophysiology in systemic sclerosis?
Vascular damage Autoimmunity Tissue fibrosis
42
Pt presents with PHTN and finger tip ulcers. What condition?
scleroderma - very important cause of PHTN, often the first presentation of the disease
43
Autoantibodies in SSc?
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
What is the pattern of skin involvement in limited cutaneous SSc?
Distal skin thickening (below elbows) - therefore if pt has diffuse skin thickening with proximal arm or face/trunk affected then this is diffuse
45
What is a key feature that almost all pts with SSc have, often before they receive the diagnosis?
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
Explain CREST?
Calcinosis Raynauds Esophageal inv Sclerodactyly Telangioectasia
47
What is systemic sclerosis sine scleroderma?
THis is systemic sclerosis with nil skin involvment - Ie pt has ILD due to SSc, but nil skin involvement
48
Pts with SSc can get cardiac involvment. What is an important manifestation of cardiac involvment?
Conduction disease due to cardiac fibrosis
49
What is the scleroderma renal crisis presentation?
This manifests as an accelerated hypertension type phenotype HTN may be asymptomatic of symptomatic. Symptoms/signs including: - Headache, visual change - Papiloedema - Cardiac failure
50
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
Developing scleroderma renal crisis Often will develop accelerated HTN, then the renal crisis
51
What medication should be avoided in SRC?
Corticosteroids
52
What is the main treatment for SRC?
ACEI/ARB
53
When I move my ankle creaks. Exam shows tendon crepitus. What condition
Systemic SSc, this is tendon involvement (tendon rub) Indicator of bad prognosis
54
What are the GI abn in SSc?
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
What are the phases of raynauds?
White, blue/purple, red
56
What distinguishes primary raynauds to secondary?
Secondary - painful raynauds (most raynauds in uncomfortable but not painful) - Other manifestation ie skin etc
57
Adult 40yo with new raynauds. Should you be concerned?
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
Abnormal nail fold capilioscopy is usually assoicated with which connective tissue disease?
systemic sclerosis
59
What is a complication of raynauds? how does it manifest and time course?
Digital ulcers - Extremely tender, last for several weeks to months - Can be very debilitating
60
What is the main risk during preganncy for systemic sclerosis?
Increased risk of Preeclapsia and ecclampsia
61
Typical treatment regime for SSc ILD?
Nintedanib (antifibrotic) and mycophenylate
62
What is mixed connective tissue disease?
Currently this is a distinct entity however the phenotypes is that of a SLE and scleroderma overlap
63
Pt with puff hands. What disease?
MCTD
64
What is the commonest cause of death (therefore the most important complication) of MCTD?
Pul HTN
65
Characteristic lab findings in MCTD? How to tell between lupus and MCTD with antibodies?
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
Sjogrens syndrome can be thought of as a disease of the ... ?
Exocrine organs
67
What are the main symptoms of SS? what is the pathological cause of these symptoms?
Sicca symptoms (xerostomia, xeropthalmous of the eye, dry vagina) - Due to lymphocytic infiltration of the exocrine glands Gladular enlargment Circulating antibodies
68
What are some DDx of parotid enlargment?
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
Palpable purpura. Dx?
Sjogrens disease
70
Pt with Sjogrens syndrome has thin walled cystic leions on HRCT. What is the Dx?
Lymphocytic interstitial pneumonia
71
Pt has lymphocytic intersitial pneumonia, what primary condition do they most likely have?
Sjogrens syndrome
72
Pt with NAGMA and Sjogrens, what is teh Dx?
Type 1 RTA
73
What is teh main renal manifestation of Sjogrens syndrome?
TYpe 1 RTA
74
Pt has mixed cryoglobulinaemia, which connective tissue disease do they have? What would be the most likely infection associated with this?
Sjogrens syndrome Hep C virus
75
What is a characteristic but uncommon neurological manifestation of Sjogrens syndrome? How does it manifest?
Sensory ganglionopathy that manifests as pseudoathetosis due to lack of proprioception in the affected limb
76
What cancer is Sjogrens syndrome most associated with?
Lymphoma (NHL, most common is MALT)
77
Autoantibodies in Sjogrens syndrome?
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
What is the antibody associated with neuromyelitis optica?
AQP4 IgG ab positive
79
What are the cardinal features of Behcets disease?
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
What HLA in Behcets disease?
HLA B 51
81
What pathological description is characteristic of sarcoidosis?
Non caseating greanulomatous inflamation
82
Lupus pernio. What condition?
Sarcoidosis?
83
What are some key rheum features of sarcoidosis? What is lofgrens syundrome?
Lupus pernio Errythema nodosum Isolated ankle arthritis Lofgrens syndrome is a clinical triad of sarcoidosis: - Fever - EN - bilateral hilar Lymphadenopathy
84
Pt has painful ears. What condition?
Relapsing polychondritis
85
What is teh main malignant associated of relapsing polychondritis?
Myelodysplasia
86
What is teh pathopneumonic histo for IgG4 disease?
Lymphocytic infiltrate Storiform fibrosis
87
What are the most common / important manifestation of IgG4 disease?
- Retroperitoneal fibrosis - Periaortitis, periarteritis (manifesting as vascular complications) - renal ionvolment

Rheumatology (4 decks)

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