RHEUMATOLOGY Flashcards

1
Q

What is Wegners Granulomatosis with polyangitis

A

Macroangiopathic vasculitis

Classically causes vasculitis in nose / airway and GN
-epistaxis
-arthropathy
-pulmonary haemorrhage
raised TLCO and kCO due to blood in airway with reduced He due to ventilation loss (restrictive picture)
-PR bleeding
-erosion of nasal septum causing saddle bridge

Diagnostic:
History
c-ANCA positive
Raised kCO and TLCO due to pulm heamorrhage

Differential:
Goodpastures - no sinus issues / epistaxis etc
churg strauss (p-anca)

Treatment:
Monitor renal function - HD or PD or transplant
plasma exchange
methyl pred+ rituximab / cyclophosphamide

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2
Q

Familial Mediterranean Fever

A

Autosomal recessive

  1. pyrexia
  2. recurrent peritonitis

assoc:
arthropathy
pleuritis / pericarditis
rash

last 2-3 days
mimics acue abdomen
laparatomy = NAD

COLCHICINE - GOOD PREVENTION
INDOMETACINE FOR PERICARDITIS

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3
Q

How should acute gout be managed

A
  1. Colchicine
    - 500mcg BD renal dose

2.Allopurinol - may worsen acute gout
good for prevention

  1. Febuxostat - xanthine ox inhibitor like allopourinol but with worse CV incidence
  2. Rasburicase - urate oxidase
    - reduce risk of gout in TLS e.g. whilst giving RCHOP
    - TREAT HYPERURICAEMIA IN TUMOUR LYSIS
    - ACCELERATES URIC ACID METABOLISM
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4
Q

What is Raynauds phenomenon

A
  1. idiopathic
  2. secondary raynauds
    - SLE
    - Sarcoid
    - RA
    - Scleroderma

Small vessel vasculitis in response to cold

Presentation: PAIN / BLUE –> RED
Peripheral vasoconstriction hands and feet leading to vlue discolouration in response to cold weather
Painful
Blanching erythema secondary to reperfusion + reperfusion injury

chronic:
CHRONIC ISCHAEMIA
Pulp atrophy
nail fold telangiectasia in response to HIF1a
arterial ulceration
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5
Q

What do you see cutaenously manifested in sarcoid

A
  1. lupus pernio
    face ears limbs
    Indurated red / black papules - cutaeneous sarcoid
  2. cutaenous non cavitating granuolmas
    - Epitheliod cells with sparce lymphocyte infiltrate marginally
    - may show calcium deposition as well
    - Sarcoid leads to hypercalcaemia due to ectopic production of 1a hydroxylase within granulomas = MORE ACTIVE VIT D3 = MORE CA ABSORPTION GUT + REDUCED EXCRETION DCT.
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6
Q

What are cold agglutinins

A

AI disorder presenting with haemolytic anaemia in response to cold weather

Abs opsonise and agglutinate resulting in
AGGLUTININ MEDIATED ACROCYANOSIS
-microocclusion
-microangiopathic haemolysis
-localised ischaemia

Warming does not stimulate reperfusion as in Raynauds.

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7
Q

What is Behcets disease?

A

Multisystem AI vasculitis
Can follow a relapsing remiting course
HLA B12/B51/B5

overlaps with chrons

  • apthous ulcers
  • abdo pain
  • seronegative arthropathy
  • weight loss

-systemic mucosal ulceration
90% oral apthous
70% genital

-Erythema Nodosum
(migratory panniculitis = inflammation of the fat cells)

  • Thrombosis
  • pathergy - abnormal scarring post trauma

20% vision loss
50% eye involvement
-anterior / posterior uveitis
-retinital vasculitis

CNS:
Can resemble MS
-sporadic lesions
-chronic meningoencephalitis.
-brainstem
4M motor N / motor p CST / MLF = ino (M+P) / med lemnisc
4S SNS (horners) / /spinothal / sensory V / cerebsopin /
(midbrain 3/4 pons 6M 578L medulla 9.10.11. L 12 M)
-basal ganglia - Parkinson plus syndrome

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8
Q

What is Jaccouds Arthropathy?
How might it present?
What is it associated with?
How is it managed?

A

Jaccouds Arthropathy
Reversible chronic non erosive deforming arthritis which typically follows repeated episodes of inflammation of the joint capsule and fibrosis that imposes traction afterwards

-ulnar deviation from mcp subluxation

ASSOCIATED

  • SLE 5%
  • POST RHEUMATIC FEVER
  • SJORGENS
  • PSORIATIC ARTHROPATHY
  • ANKYLOSING SPONDYLITIS
  • MIXED CONNECTIVE TISSUE DISEASE
MX:
PAIN CONTROL
physio
Osteotomy
Kirschner intramedullary wire for stability
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9
Q

What is Rheumatoid arthritis?

A

A common (1% of pop) autoimmune erosive symmetrical small and large joint arthropathy typically sparing the DIPJ.

It is set apart from other seronegative joint arthropathys like ankylosing spondylitis which affects primarily larger joints

It shares similarities with psoriatic arthropathy which has a typically varied presentation

The focus is on early diagnosis and early intervention to

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10
Q

How is Rheumatoid arthritis diagnosed?

Systemic signs

Scoring mech: - Im knacked (NACD)
NACD

A

Joint involvement

  • Symmetrical
  • Small and large joints
  • Early morning stiffness
  • RA rubbery nodules
  • ulnar deviation of the MCP joints
  • small tender effusions
  • boutonnieres deformity - tendon sheath ruptute
  • Swan neck - pipj and dipj tendon sheath rupture
  • Z thumb

Neuro
Cervical myelopathy - Atlanto-axial subluxation = snap peg
Ulnar neuropathy - claw - paradox - more prox lesion worse deformity
Carpal Tunnel syndrome

SURGICAL SCARS
Swansons arthrodesis of MCPJs
Carpal Tunnel decompression
Wrist Arthrodesis
Ulnar styloidectomy
Wrist synovectomy
TENDON TRANSFER - follows line of tendon

EXAMINE FOR SPLENOMEGALLY / ANAEMIA
-Felty’s syndrome - splenomegally/ neutropenia / RA

RESP
pulmonary fiborsis
- secondary to RA or MTX

Renal
Amyloid

SCORING MECH - >6 = RA
NACD = number / Abs / CRP / duration

-Number of joints involved
large = 1
1-3 small =2
4-10 small = 3
>10 = 5

-Serology:
High RF titre + Anti-CCP positive–3
Low RF titre + Anti-CCP positive–2

  • CRP +ESR = 1
  • Duration of symptoms >6/52 = 1
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11
Q

How is RA treated

DMARDS
=MLSH
Biologics
=T(3 targets) 
=B
A

Aim is for early diagnosis and early treatment to induce remission and a state of disease quiescence

Physiotherapy to maintain mobility and ROM

DMARDS

  • MTX
  • Sulfasalazine
  • Leflunomide
  • Hydroxychloroquine

Biologics:
Anti-TNFa - INFLIXIMAB / ADALIMUMAB / GOLIMUMAB
TNF-R ANTAG - ETANERCEPT
PEG-FAB VS TNF-A- CERTILUZIMAB PEGOL

RITUXIMAB - ANTI-CD20 (no B cell Ab production)
ABATACEPT - CTLA-4 AGONIST (i Tcell acitvation)
TOCILIZUMAB - ANTI IL-6 - reduces TNF-a

Surgery - uncontrolled disease or disability

  • arthrodesis - fusion
  • arthroplasty - replacement

Analgesia

finger braces

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12
Q

Why is RA associated with anaemia

A
  1. Anaemia of chronic disease
  2. MTX
  3. UGIB secondary to NSAID use

FELTYS SYNDROME
RA / NEUTROPENIA / SPLENOMEGALLY

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13
Q

What are the side effects of MTX

A

Counselling needed

  • pancytopenia
  • hepatitis
  • pulm fibrosis
  • stomatitis
  • anaemia

MOA:
Blockade of folic acid metabolic pathway / IL-1 inhibition / DNA synthesis / purine metabolism / thymidine synthesis / RNA synthesis

immunosuppression and opportunistic infection
need regular blood test to look for pancytopenia and trans-aminitis

Treat toxicity
FOLINIC ACID

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14
Q

MOA of MTX
CANCER?
RA?

A

Methotrexate is an antimetabolite of the antifolate type.

CANCER: HIGH DOSE
= antimetabolite / no purines / reduce DNA RNA synth

methotrexate competitively inhibits dihydrofolate reductase (DHFR) (binds folate)

DHFR catalyses the conversion of dihydrofolate to the active tetrahydrofolate for the de novo synthesis:

  • nucleoside thymidine–>required for DNA synthesis
  • folate is essential for purine and pyrimidine base biosynthesis,

Methotrexate, therefore, inhibits the synthesis of DNA, RNA, thymidylates, and proteins

RA: LOW DOSE
= TURN OFF Bcells / reduce costim / reduce IL-1 sensitivity

  • inhibition purine metabolism, leading to accumulation of adenosine;
  • inhibition of T cell activation and suppression of intercellular adhesion molecule expression by T cells;
  • selective down-regulation of B cells; increasing CD95 sensitivity of activated T cells;
  • inhibition of IL-1 to its cell surface receptor
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15
Q

What are the signs of Psoriatic Arthritis

A

Small and large joint SERO NEGATIVE + ASYMMETRICAL oligoarthropathy with a variable presentation associated with psoriatic skin changes preceding or developing after arthropathy onset

This develops in 5-10% of people with psoriasis

70% have psoriasis for >10 years before onset of arthritis
30% arthritis onset is before or at the same time
More common with nail involvement

onset 30-50
1st deg FMH = 50 fold increase risk
father PsA = risk x2 to child
male = female

Presentation:

  1. psoritic papular plaques - extensors + Koebner
  2. back pain - ASYMMETRICAL spondyloarthropathy - commoner men
    - Symmetrical favours ANK SPOND
  3. PR bleeding / altered bowel habit
    - ASSOC CD + UC
  4. Iritis
  5. Achilles Tendonitis
  6. Arthtiris mutilans / dipj / symmetrical small joint RA like /oligoarthropathy + effusion
  7. Dactylitis
  8. BILATERAL PLANTAR FASCIITIS
  9. NAILS
    - pitting / onycholysis / transverse ridging / flaking /yellowing /
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16
Q

What are the 5 patterns of joint involvement in PsA
What are the HLA associations

RADOS

A

PsA is a SERONEGATIVE oligoarthropathy which typically causes large joint effusion and dactylitis

There are 5 types of presentation

1) RA like - symmetrical / persistently seronegative
- HLA DR4 (also CD)

2) ARTHRITIS MUTILANS
- very deforming
- joint telescoping
- joint destruction and finger shortening
- Dactylitis

3)DIPJ involvement
HLA B38 and 39

4)Oligoarthritis - asymmetrical + MASSIVE EFFUSIONS

5)Spondyloarthropathy - like ank spond but ASYMMETRICAL
HLA B27

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17
Q

How is PsA treated

A

Analgesia

Avoid steroids - acute flare on withdrawal

DMARDS 
MTX - mainstay
leflunomide - joints and skin
sulfasalazine - little skin effect
hydroxychloroquine - AVOID AS MAKES SKIN WORSE

ACHILLES TENDINOPATHY
-Steroid injections = very good

Biologics
-Anti Tnf - inflix / adalimumab / golimumab = joint + skin

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18
Q

Joint differentials

A

DIPJ - HLA B38 AND 39
NOT RA
PsA - subtype
OA - erosive

Small joint symmetrical arthritis - HLA DR4
RA
PsA subtye RA like

Dactylitis - sausage fingers
SARCOID
Gout
PsA
Reactive arthritits

Explosive psoriasis + Joint involvement
-loss of Tregs - CD45 - think HIV

Spondyloarthropathy

  • Ank spond
  • PsA - HLA B27 TYPE
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19
Q

How can you differentiate PsA from RA

A

-RA may be sero positive - PsA always sero-negative
low or high RF
positive anti-ccp

  • Cutaneous psoriatic plaques and pitting in PsA
  • PsA Dactylitis - entire finger or toe inflammed
  • DIPJ involvement in PsA subtype HLAB38 and 39

-Arthritis Mutilans PsA
telescoping
shortening
severe erosive arthritis

-RA symmetrical - PsA except RA type asymmetrical

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20
Q

How can you differentiate OSA from RA in xray

A

BOTH
narrowed joint space
bone cysts

RA
symmetrical joint involvement
subluxation
juxta-articular osteopenia
erosive arthropathy

OSA
osteophytes
osteosclerosis

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21
Q

What is SLE?

A

A common multi-organ involving auto immune ANA positive vasculitis which may present with arthropathy, vasculitic sequelae , glomerulonephritis and cutaneous features

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22
Q

What are the erosive arthropathies?

A

RA
PsA
Erosive OA

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23
Q

What are the non erosive arthropathies?

A

SLE arthropathy

Jaccouds

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24
Q

What are ACR criteria for diagnosing SLE?

RASH GANA

ANA for antibody most specific neuro and arthropathy

Rash 3
Apthous ulcers 1
Serositis 1
Haemolytic anaemia / leukopenia etc 1

GN 1
Antibodies 2
Neuro 1
Arthropathy 1

A

NEED 4 or more = 4/11

Malar Rash
Discoid Rash
Photosensitivity
Apthous ulcers
Non erosive arthritis - JACCOUDS
Serositis - pleural effusion / pericarditis
Glomerulonephritis
Neuro SLE = GBS / seizures / psychosis / T myelitis / INO/ mononeuritis multiplex
Haem = haemolytic anaemia / leucopenia /low plt
ANA positive - 95% >1:320
Anti-phospholipid (stroke tia mi retinal art / Anti - DsDNA - good for flares. low c3 c4

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25
Q

How might SLE present

Think:
Multi system

Cutaneous SLE.
Neuropsychiatric SLE.
GN SLE ? 
transplanted / PD scars?
Heart failure - libman sacks endocarditis

Assoc:
Autoimmune ovaarian failure - secondary amenorhhoea
recurrent miscarriages -antiphospholipid

A

-Arthritis
non deforming jaccouds arthritis

-alopecia + mouth ulcers
scarring or non scarring

-opthalmoplegia or CN lesions
mononeuritis multiplex

-Malar butterfly rash or systemic discoid rash

-Haematuria
Lupus glomerulonephritis

-VTE + PE / stroke / MI / Retinal A occlusion early onset
Antiphospholipid syndrome
RECURRENT MISCARRIAGE + PRE-ECLAMPSIA

-Anaemia
haemolytic anaemia / leucopenia / thrombocytopenia

-Cardiac
NON INFECTIVE ENDOCARDITIS
LIBMAN SACKS ENDOCARDITIS - AR MR
CCF

-Neuro
Psychosis / GBS / seizures

-Myositis
MIXED CONNECTIVE TISSUE DISEASE OVERLAP

- SOB
Pericarditis 
Pleural effusion (transudative)
PE
pulm fibrosis
GBS
Reaction to biologics - as ARDS

-Cushingoid
steroids

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26
Q

Causes of pulm fibrosis

VITAMIN C

A

APICAL

work:
silicosis
coal pneumoconiosis

Autoimmune
Sarcoid and other connective tissue disease
ank spond

infective
Allergic aspergillosis
TB

metabolic
Histiocytosis X

iatrogenic
Radiation

BASAL

Autoimmune
RA
MCD - sjorgens / SLE / scleroderma / dermatomyositis / polymyositis

Idopathic
IPF - AIP / DIP /

infective
bronchiectasis

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27
Q

What is Sjorgens Syndrome

A

Primary or Secondary - usually Secondary
AI process which destroys exocrine glands
Usually secondary to another AI process like RA or SLE

Primary Sjorgens is associated with a 40fold increased risk of Lymphoma

Due to parotid involvement differentials of swelling should include:

  • Parotid cancer
  • Lymphoma
  • Sjorgens
  • Mumps - parotitis
  • sarcoid
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28
Q

How Does Sjorgens present

A
Dry eyes - XEROPHTHALMIA
Dry Mouth - XEROSTOMA
Bilateral parotid enlargement (blocked)
-LOOK FOR EXCISION SCARS UNDER JAW LINE
non erosive small joint arthropathy
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29
Q

What is Shirmers Test and how is it performed?

A

Test for lacrimation when sjorgens suspected

litmus paper crimped and hooked inside lower eyelid for for 5mm

expect to see tear migration >15mm = normal

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30
Q

How is Sjorgens diagnosed

A
  1. History
  2. Hx of CTD - Ra or SLE
  3. Abs - anti Ro / La

RF and anti-Ds DNA may also be positive

  1. Salivary gland biopsy is conclusive
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31
Q

What is the antibody profile in SLE?

A

Anti nuclear antigen is +ve in 95% at >1:320

Anti phospholipid may be present
Anti-Ds DNA if present good for monitoring activity
Anti-ENA may also be present
-Sm / Ro / La (THINK IS 2dary SJORGENS PRESENT)

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32
Q

How is SLE managed?

A
  1. Pred long term - may be cushingoid as in RA / PsA
    - steroid induced DM2
2.DMARDS
MTX
hydroxychloroquine
MMF
Cyclophosphamide (esp lupus nephritis)

3, biologics
Rituximab - anti cd20
IVIg for flares
plasmaphoresis

  1. anticoag if antiphospholipid
    Warf - antag vit K
    Rivarox / apix /
  2. renal biopsy
    ACEi for protein urea
    transplant
  3. TAVI
  4. iron for haemolytic anaemia
  5. physio for jaccouds arthropathy
  6. specialist
    stroke
    mi / vte
    pulm fibrosis - apical OR if secondary to MTX basal
    pleural effusion - Percutaenous drain / pleurodesis
    Pericardial effusion - pericardiocentesis

10 cosmetic
Wigs if scarring alopecia

  1. counselling
    support groups
    PT and OT input
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33
Q

How would you counsel a patient with SLE re: children

A

Conveying exact genes for SLE is rare
increased risk compared to background population

medication counselling
STOP MTX as teratogenic
STOP WARF as teratogenic
STOP Carbemazepine as teratogenic

azathioprine and hydroxychloroqione ok

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34
Q

How can you monitor SLE activity?

A
  1. Urine - protein and blood / casts / biopsy
  2. Anti-Ds DNA - levels correlate to disease activity
  3. FBC - cytopenias
  4. LFTs - Transaminitis
  5. Low C3 low C4
  6. haematuria
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35
Q

What are the risks associated with cyclophosphamide?

A

1 Immunosuppresion

Reactivation of latent TB / HCV / HBV / CMV / EBV
Acceleration of undiagnosed HIV

Opportunistic infection due to BM suppression
PCP
CMV or EBV or HTLV-1

THEREFORE PROPHYLACTIC COTRIMOX

2.Small risk of increased cancer.

  1. Hemorrhagic cystitis
    TAKE ORAL MESNA PRIOR TO INFUSION
    Conjugates toxic urinary metabolites to reduce the risk.
  2. infertility
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36
Q

What is systemic sclerosis? How is it different from Scleroderma? Can scleroderma present in any other way?

A

Systemic form of scleroderma
Multi organ involving CTD characterised by skin thickening and joint deformity

CREST no longer really applies therefore:

  1. systemic sclerosis - CREST
  2. limited cutanoues - not all CREST fulfilled - e.g. gastroenteropathy + pulm fibrosis + morphoea

Look for

  • small tight mouth / beaked nose
  • tight shiny skin
  • sclerodactyly deformity
  • morphoea - Limited cutaenous SS or DcSS
  • calcium deposition - CREST
  • ulcers over fingers - raynauds
  • hair loss and scarring alopecia
  • telangiectasia (CREST)

artificial tears / saliva = Sjorgens

Remember CTD do cross over
-SLE may also feature here
(RASH G-ANA) rash malar discoid photo / apthous / serositis / haemolytic / GN / ANA / Abs / Jaccouds arth)
-E.g MCTD - SLE + scleroderma + myositis

3 presentations:

Raynauds

Localised scleroderma aka morphoea
linear scleroderma well circumscribed
Tight hard skin
en coup de sabre - scalp scerloderma

Systemic Sclerosis - 3 types

-Limited cutaeonus systemic sclerosis =any non CREST
Diffuse morphoea face / below elbows and knees but spares trunk - typically get pulm fibrosis and gastroenteropathy

Pulm fibrosis types - usually SCL70 +VE

  • CREST - term no longer used (true systemic sclerosis)
    Calcinosis - isolated hypercalcaemic deposits not unlike sarcoid granulomas (due to ectopic a1 hydroxylase production)
    Raynauds
    Eosphagial dysmotility / dilatation / achalasia
    Scleroderma / sclerodactyly
    Telangiectasia

Dysmotiity extends to all bowel - bacterial overgrowth / malabsorption / weight loss and diarrhoea

pulmonary fibrosis -->p.HTN and CCF
UIP - usual int pneumonia = crap - 2.5-5 years
NSIP = non specific int pneumonia
GOOD STEROID RESPONSE 
MORT 12-14 years

-Diffuse cutaenous systemic sclerosis
Whole body morphoea
SCL70 +VE - risk of severe ILD
Anti-RNA pol 3
RISK OF SCLERODERMA RENAL CRISIS
- in event of renal crisis treat BP with RAMIPRIL - Better outcome mortality - 76% survival at 1 year Vs 15%
- amlodipine is good for BP control regarding raynauds but does not alter mortality in renal crisis

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37
Q

What is Scerlodactyly?

A

Localised thickening of fingers and toes DISTAL TO MCPJ

INVOLVEMENT POST MCP IS BEYOND SCLERODACTYLLY AND IMPLIES MORE DIFFUSE INVOLVEMENT

38
Q
specific Questions for specific CTD
SLE questions
Antiphos question
Sjorgens question
CREST question
sceroderma Qs
A
SLE (RASH GANA)
rash in the sun? Do you have any rashes?
Joint pains - jaccouds
SOB / chest pain
Haematuria
mononeuritis multiplex / gbs /psychosis / confusion
apthous ulcers

Antiphos
stroke / mi / PE / VTE

DM
rash - heliotropic
muscle weakness / pain?
Features of SLE and Slceroderma

Sjorgens
dry mouth
dry eyes
joint pains - jaccouds

CREST
Skin changes when cold ? painful? ?ulcers?
Do you have difficulty swallowing / heartburn?
DIARRHOEA /DYSPHAGIA / DYSPEPSIA

39
Q

What is scleroderma crisis

A

ONSET IN DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS

  • associated Anti RNA pol 3
  • associated with SCL70 = diffuse ILD
  • Acute onset malignant HTN leading to acute renal failure / headache // blurred vision , seizures, CCF AMD microangiopathic haemolysis
40
Q

How does dermatomyositis present?

A

Autoimmune process and associated with , CREST, SLE, malignancy (breast lung gynae / colorectal)

Causes degernation of collagen resulting in

  • heliotropic (surrounding eyes) rash
  • proximal muscle weakness
  • GOTTRON’s PAPULES over DIPJ / MCPJ - violaceous papules

Abs:
Anti Jo

onset 40-60
more common in women

41
Q

Atopic Eczema management

A
  1. topical steroids
  2. anti-histamines
  3. daily emollients
  4. topical calciuneurin inhibitors for refractive disease
42
Q

RA disease treatment in pregnancy

A

DMARDS
MTX - terato
Leflunomide - terato
azathioprine - shown to be safe but not recommended

SULFASALAZINE and HYDROXYCHLOROQUIN are the current recommendation

TNF-a inhibitors - inflix / adalimumba and golimumab have been shown to be safe but are not yet recommended due to lack of longterm evidence

43
Q

How do you treat MTX toxicitiy

A
  • glossitis
  • candidiasis
  • macrocytic anaemia
  • thrombocytopenia
  • severe mouth ulcers

–> FOLINIC ACID
More efficacious than folic acid
75mg over 12 hours
6-12mg every 4 hours thereafter

If severe
Bicarbonate
fluids

Dialysis does not remove mtx

44
Q

What are the diagnostic criteria for FIBROMYLAGIA

1990 ACR

A

Widespread pain for >3 months
Pain elicited by pressure of 4kg/cm2 in 11 areas
Psychological stress = > pressure points

Overlaps substantially with depressive disorders / anxiety and chronic fatigue
overlaps with regional pain syndromes - atypical CP / IBS / chronic pelvic pain / TMJ pain

45
Q

How is Fibromyalgia treated

A
  1. psychotherapy
  2. pharmacotherapy for disordered sleep = tricyclics / melatonin / zopiclone
  3. graded exercise programs
46
Q

What is Gout?

A

Polyarticular or monoarticular gout

  • single or multiple hot effused joints
  • restricted ROM due to pain
  • attacks last 3-10 days
  • first presentation monoarticular - big toe / ankle / foot arch
  • 50% onset hallux–>90% involvement
  • Diurnal variation - onset in night and worst in morning
  • Olecranon bursitis
  • scars from ulceration and sinus formation over joints

-Aspiration - needle shaped intracellular and extracellular crysals of monodosium urate
yellow crystals–>red aligned parrallel–>blue when aligned perperndicular (NEGATIVE BIREFRINGENCE)

PSEUDOGOUT:
Calcium pyrophosphate (CPP) - short / rhomboidal - no colour change underpolarising filter
TOPHI:
ACHILLES
BEHIND PINNA
ELBOWS
FINGERS / TOES
EXTENSOR FOREARM ALONG TENDONS

PODAGRA - foot torturer in greek mythology
HALLUX GOUT

Caused by precipitation of urate crystals within the joint space causing localised inflammation and pain with restricted movement

Gouty arthritis is the most common for of arthritis in men over 40

4x more common in men

Prevelance rises with age and serum urate concentrations

4 PHASES
1 Asymptomatic Hyperuricaemia
2 Acute Gout
3 Intercritical Gout
4 Chronic tophaceous gout
triggers:
alcohol
purine ingestion (shellfish)- concurrent azathioprine use will require AZA reduced dose will need to be considered to maintain IBD control and avoid excessive BM suppresion
UGIB and ingestion of digested blood
sepsis
trauma
dehydration
drugs:
TZDs  - LOW POTASSIUM
aspirin
theophyllin
ciclosporin
levodopa
ethambutol
Risk factors
CKD
hypothyroid
myeloprolif / lymphoprolife: high turnover
DM
DI
hereditary hyperuriacaemia
Sarcoid
Psoriasis
obesity

Allopurinol is a xanthine oxidase inhibitor which would normally facilitate breakdown of 6’mercaptopurine (active form of AZA and mercaptopurine) (also inhibits TPMT) - Therefore in IBD can use a low dose of thiopurine analogue and supplement wit allopurinol to have clinical effect

Mx:
Colchicine / pred / NSAIDs (naproxen NOT IBUPROFEN) in the acute setting
Allopurinol or febuxostat (XOI) after symptoms resolve for secondary prevention
RASBURICASE - recombinatn urate oxidase
Avoid precipitants - alcohol / caffeine / cheese

47
Q

How is Gout best managed?

A

Mx:
Colchicine / pred / NSAIDs (naproxen NOT IBUPROFEN) in the acute setting
Allopurinol or febuxostat (XOI) after symptoms resolve for secondary prevention
RASBURICASE - recombinatn urate oxidase
Avoid precipitants - alcohol / caffeine / cheese

48
Q

When are Gottron’s papules seen?

A

Indicative of dermatomyositis

49
Q

What is Reactive Arthritis?

A

Spondyloarthritis presenting with triad:
urethritis / dysuria
uveitis
arthritis

Causes
E.Coli / shigella / salmonella Gastroenteritis
Chlamydia trachomatis
Chlamydia pneumoniae

Psoriatic like rash = chlamydia

Treat = cause

50
Q

What is Carpal Tunnel Syndrome?

What conditions are associated with it?

A

CTS results from median nerve compression in the wrist affecting the median nerve distribution

  • Thumb movements except flexor pollicis brevis
  • thenar wasting
  • 1st and 2nd fingers
  • Tinnels and phalens sign positive
Associations:
RA
Hyperthyroid
Hypothyroid
Idopathic

Mx:
local consticosteroid
hand splint
median nerve decompression

EMG studies to confirm

51
Q

What is seen on aspiration in Gout?

A

Needle shaped negative birefringence crystals
yellow at base
aligned to light = red
perpendicular to light = blue

WCC +++
no growth

52
Q

What is seen on aspiration in pseudogout?

A

calcium pyrophosphate crystals

no birefringence

53
Q

What is seen in HPOA

A

Clubbing

periosteal inflammation i.e. effusion similar to dactylytis but involves whole fingers

54
Q

What is HPOA?

A

Hypertrophic pulmonary osteoarthropathy

55
Q

How does osteomalacia present?

A

global joint pain

proximal weakness

56
Q

What is Charcot Arthropathy?

A

Results in Charcot’s joint

  • TYPICALLY PAINLESS DUE TO SENSORY PARAESTHESIA AND IMPAIRED PROPRIOCEPTIVE FEEDBACK
  • GROSS DEFORMITY

XRAY

  • loss of joint margin
  • bony destruction
  • significant and active remodelling

Rx:
immobilisation for 3-4 months
bisphosphonates
IRREVERSIBLE ONCE ESTABLISHED

Causes:
DM
Charcot Marie tooth
- hereditary sensory and motor neuropathy
- demyelinating / schwann cell damage
- loss of sensation
- pes cavus or pes planus
- CMT is divided into the primary demyelinating neuropathies (CMT1, CMT3, and CMT4) and the primary axonal neuropathies (CMT2), with frequent overlap.
57
Q

What is allopurinol’s effect on azathioprine?

A

Allopurinol is a xanthane oxidase inhibitor
XO converts 6 mercaptopurine to inactive metabolites
Inhibition of XO results in build up of active component 6 mercaptopurine
i.e. dose seen by tissue is much greater than dose prescribed.

58
Q

What antibodies are present in mixed CTD

A

Anti-RNP

59
Q

How does Cryoglobulinaemia present

A

Immunoglobulins that precipitate out of solution when exposed to cold temperatures causing microvascular complications

  • purpuric rash
  • polyarthralgia
  • polyarthritis
  • Raynauds

Abs:
ANA
RF - 90% present in Sjorgens and cryoglobulinaemia
low C3 - immune complex deposition

60
Q

How can Pulmonary HTN be reduced in Sarcoidosis

A

Remember pulmonary sarcoid will cause bilateral hilar lymphadenopathy and basal pulmonary fibrosis

P.HTN is a significant cause of morbidity and MORTALITY
-core pulmonale
severe  = PA pressure >40mmHg
-PR
-TR
-peripheral oedema
-OSA
-T2RF
-impaired ET

Treatment for sarcoid = improve QOL and slow progression

  1. Pulmonary rehab
  2. stop smoking
  3. Sildenafil to reduce Preload
  4. iloprost infusion

Longterm:
Pred
MTX

61
Q

Why is arthritis associated with Hereditary Haemochormatosis

A
Presentation:
40M
Skin pigmentation
Diabetes
Heart problems
CLD
Arthropathy
Arthropathy is due to CHONDROCALCINOSIS
CALCIUM PYROPHOSPHATE DEPOSITION
i.e. pseudo gout / chondrocalcinosis / chronic arthropathy
Rhomboid crystals
may be weakly positive birefringence

-High transferrin saturation >15%
raised serum ferritin >500

62
Q

In PsA WHERE might you find hidden psoriasis?

A

scalp i.e hairline
gluteal cleft
naval

flexor tendon synovitis

63
Q

How does hydroxyapatite arthritis present

A

Usually female
Usually monoarthritis
Usually glenohumeral joint

aspirate:
blood
hydroxyapatite crystals

VERY DESTRUCTIVE ARTHRITIS

64
Q

What DMARD for RA is safe in pregnancy? What does NICE recommend?

A

Treatment should continue through pregnancy especially given the immuno changes that come i.e. tolerance

MTX / Leflunomide = teratogenic
Sulfasalazine = FIRST CHOICE
Azathioprine = Safe but not first choice
Infliximab = data shows no increased morb / mort but not yet approved by NICE for pregnancy

65
Q

What is Felty’s Syndrome?

How does it present and how is it managed?

A

RA + splenomegally +neutropenia
50s
female
longstanding RA >10 years

Think as a differential in a patient with RA on MTX
i.e. not simply MTX tox = folinic acid resuce

Rx:
pulse methylpred
cyclophos
switch DMARD - leflunomide / sulfasalazine / azathioprine
SPLENECTOMY IF FAILS TO RESPOND
66
Q

What is bronchopulmonary aspergillosis and what are its sequelae?

A

Manifests as EEA

  • Driver is allergen to aspergillus
  • Raised eosinophil count - Th2 response
  • Raised aspergillus IgG
Aspergillus can seed in old TB cavities
Can become invasive aspergillus
Pulmonary haemorrhage
Form a gravity dependent aspergilloma
pulmonary fibrosis - apical
Can also cause bronchiectasis due to chronic poor drainage

(CAUSES Bronchiectasis = TONNES - CONGENITAL = CF / YOUNGS (middle Age + bronchiectasis, rhinosinusitis and reduced fertility. / A1 ANTI-TRYPSIN / Kartageners - Primary ciliary dyskinesia / TOXIN - Recurrent aspiration / COPD / Traction bronchiectasis (pulmonary fibrosis splints open airay) / IMMUNO - HIV +LEUKAEMIA +SCID /MECHANICAL - obstruction / carcinoma / granuloma - sarcoid wegners tb )

Treatment 
Fostair - formeterol /  fluticasone OR
Seretide - salmeterol / fluticasone
Pred for wheeze if active
ITRACONAZOLE
pulmonary rehab

SLOW PROGRESSIVE IN MOST CASES

67
Q

What is the greatest adjuvant to smoking cessation?

A

BUPRIOPION AND VARENICLINE
single agent = 20-30% cessation
double agent up to 44% cessation

Willpower alone = 2-4% cessation

nicotine replacement lozenges / gum = 10%

68
Q

How might Pneumocystitis Jirovecci present on CXR

A

Bilateral crackles
pyrexia and T1RF
leukopenia

CXR:
interstitial shadowing
basal cystic changes

CYSTS AS ANY BULLAE TYPE DISEASE AN RUPTURE CAUSING PTX - 10%

69
Q

How is PCP diagnosed?

A

AIDS defining illness
CD4<200
also:
SCID / any immunosuppressed

Diagnosis:
History
CXR - interstitial shadowing + cystic lower zone changes
BAL - fungus
immunofluorescence and BAL
70
Q

How is PCP Treated?

A

long term prophylaxis:
Co-trimoxazole
HIV ART/HAART compliance and CD4>200

Acute:
Prednisolone
Co-trimoxazole

71
Q

What joints are particularly at risk to AVN

A

Humeral head –> almost all humeral neck fractures
Femoral head –> intracapital fractures
Scaphoid

SLE presentation
Chronic and intractable hip pain
90% progress to bilateral <12 months

Antiphospholipid
Gauchers storage disease

MRI best modality

Mx:
non weight bearing to prevent the colpase of the the femoral head and secondary OA
Surgical deompression
Hemi Arthroplasty

72
Q

How is osteoporosis managed

A

T Score

73
Q

How does gonococcal arthritis present?

A

dermatitis / polyarthritis / tenosynovitis / urethritis

Pustulous eruptions
Scaly pustular plaques
nail pitting
single joint disease above others - hot swollen (septic joint)

Mx:
Ceftriaxone
Azithromycin
NSAIDs

74
Q

What is first line for septic arthritis?

A

Benzylpenicillin and flucloxacillin

75
Q

Drug induced lupus

A
commonest manifestation
Cutaeneous rash which is photosensitive
\+VE ANTI-HISTONE ABS
\+VE ANA
\+VE RF 
-VE ds DNA
Normal C3 and C4

i.e appears like inactive lupus but cutaenous photsensitivity

causes:
HYDRALAZINE
SULFASALAZINE
CAPTOPRIL

RX:
low dose steroid
stop drug

76
Q

What antibody is associated with CTD

A

Anti-RNP antibodies

77
Q

How does Wegners Granulomatosis with Polyangitis present and what is the initial management

A

Granulomatous and necrotising systemic vasculitis of medium and small calibre vessles not associated with raised eosinophils.

Presentation:
Necrosis of nasal septum and bridge = SADDLE BRIDGE
Epistaxis
Pulmonary Haemorrhage
GN
Cavitating chest lesions

C-ANCA positive PR-3
(CHURG STRAUSS + microscopic polyangitis =P-ANCA MPO)

Immediate Mx:
Pulsed IV methyl pred + cyclophosphamide / ritux
Biologics:
Rituxumab - non inferior to cyclophosphamide

Remission rate is only 75%

Chronic:
Oral pred + steroid sparing DMARD
Steroid bridges to get steroid sparing agent to a therapeutic level
e.g. MTX
Azathioprine or Mercaptopurine (need TPMT level - low TPMT = avoid)
Infliximab
Prophylactic co-trimoxazole

78
Q

How Can UC and CD be differentiated based on ANCA?

A

80% UC is positive for atypicaly ANCA - ANCA-X Vs 20% of CD

79
Q

What is ANCA

A

Antineutrophil cytoplasmic antibodies
Abs directed against neutrophils

C and P denote where Ab found
C = cytoplasm
P = perinuclear

MPO or PR3 are the targets

C-ANCA is always targeted at PR3

P-ANCA is always targeted at MPO

80
Q

What do ECG leads correspond to?

A

1 LAT AVR V1 septal V4 ant
circumflex LAD RCA
High Lat LV ant/septal Ant LV

2 INF AVL: LAT V2 septal V5 Lat
RCA circumflex LAD circumflex
INF LV High Lat LV ant/septal Lat LV

3 INF AVF: INF V3 ant V6 Lat
RCA RCA RCA circumflex
INF LV INF LV Ant LV Lat LV

81
Q

Signs of AS?

A
Carotid upstroke
LVH and heave
Reversed splitting on second heart sound - delayed A2
syncope = severe 
SOBOE
poor pulse volume
syncope on exertion
SVT  / Vt secondary to LVD
82
Q

How can Aortic valve status be confirmed post ECHO in LVF

A

LVF may cause an underestimate of the valve pressure gradient and valve surface due to impaired contraction and impaired force of contraction(FALSE LOW)

For this reason it is better to go on : DOBUTAMINE STRESS ECHO
Severe AS = rise in trans-aortic pressure on dobutamine stimulation with same valve area i.e. a true estimate

If the pressure falls and valve area increases then estimate was likely a false low estimate

An increase in EJF of over 20% is associated with a much better prognosis post TAVI

83
Q

What is morphoea?

What is it associated with?

A

Morphoea is a localised area of skin thickening which can be isolated or form part of the manifestation of limited or diffuse systemic sclersis or CREST

(calcinosis / raynauds (ANA / RO / LA) / GI / sclerodact / telangiectasia)

Typically linear thick plaques that become atrophic
hypo or hyperpigmented
over new damaged tissue
tight and itchy skin
on head - morphoea en coup de sabre
white striae

Mx:
topical or oral steroid
phototherapy
MTX

84
Q

What anti-bodies are associated with Dermatomyositis?

A

Anti-jo

onset 40-60
2:1 F
associated cancer - lung breast uterine stomach rectum
-precedes cancer 40%
-concurrent 20%
-30% post neoplasm
  • Gottrons papules
  • heliotropic rash
85
Q

What antibody is likely positive in CREST with renal involvement?

A

Anti-RN pol III

increased risk of scleroderma renal crisis
REDUCED RISK PULM FIBROSIS

Anti-SCL70

increased risk of PULM FIBROSIS

Anti-topoisomerase -1
ILD + renal involvement

86
Q

What is anti-topoisomerase-1 ab associated with?

A

systemic sclerosis

ILD and renal involvement

87
Q

What is anti-centromere antibody associated with

A

limited cutaenous sclerosis
RF for P.HTN

NOTE ILD AND RENAL INVOVLEMENT VERY RARE IN LIMITED DISEASE

88
Q

What reduces mortality in scleroderma renal crisis

A

Ramipirl

76% survival at 1 year Vs 15% without

89
Q

What is of symptomatic benefit in treatment of raynauds

A

Amlodipine

90
Q

How is lupus nephritis treated?

A

Prednisolone + cyclophosphamide or mycophenolate

Rtiux no use
MTX onset too slow

91
Q

How is aknylosing spondylitis managed?

A

Regular analgesia and pain relief
physio and mobiisation
Atni-TNFa = ETANERCEPT / ADALIMUMAB / GOLIMUMAB
STEROID INJECTIONS for sacroilitis and enthetesis

Bisphosphonates for osteoporosis