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RACP > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

RF for hydralazine drug induced lupus

A
  • high dose >200mg
  • slow acetylator
  • HLA-DRw4
  • > 3month of therapy
  • Female
  • FHx of autoimmune
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2
Q

Antibodies associated with anti synthatase sx

A

Anti-Jo1
Anti PL-7
Anti PL 12

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3
Q

scleromalacia perforans

A

Anterior necrotizing scleritis without inflammation, so called scleromalacia perforans, is a rare, severe eye disorder developing an autoimmune damage of episcleral and scleral performing vessels (hypersensitivity type III)

Associated with GPA and also RA

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4
Q

RA

A

Rheumatoid nodules are commonly seen in seropositive patients treated with Methotrexate alone.

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5
Q

Which clinical features of GCA is associated with positive biopsy?

A

Jaw claudication

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6
Q

GCA

A

Genetic association:
- IL17A, IL33, HLA DRB104

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7
Q

Which group of agents should we not precribe colchicine with due to increased risk of SE?

A

Strong CYP3A4 and P-gp inhibitors
amiodarone, cyclosporin, digoxin
diltiazem, antifungals such as itraconazole, macrolide antibiotics, statins and fibrates

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8
Q

What shoulder patholgy exhibits the painful arc?

A

Supraspinatus tendonitis

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9
Q

Secukinumab

A

IL17a inhibitor used in the treatment of ankylosing spondylitis and associated arthropathy. It has effect on both peripheral and axial arthritis and appears to slow radiographic progression. It is unclear whether this drug can worsen inflammatory bowel disease and therefore the current recommendation is to avoid using this drug during IBD flares. Secukinumab has some benefit in improving psoriasis

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10
Q

What kind of ILD is RA more like?

A

UIP

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11
Q

Belimumab

A

Belimumab is a human monoclonal antibody that inhibits B-cell activating factor, also known as B-lymphocyte stimulator. It causes reduction in B cell survival instead of depletion (which is what Rituximab causes)

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12
Q

Histological features of GCA

A

Panarteritis (CD4 Lt, histiocytes, plasma cells)
Giant cell granuloma
Distruption to the internal lamina (not specific)
Patchy and skin lesions
Thrombosed and stenosed vessels

Disruption of the internal elastic lamina is a hallmark feature and tends to persist even after steroid treatment.

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13
Q

If you have GCA and your vision is threatened, what is the treatment?

A

Methylpred

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14
Q

Does the use of OCP increase or decrease the risk of RA?

A

Decrease (esp those who are CCP positive)

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15
Q

Factors that increase the risk of lymphoma in those with sjogren’s disease

A
  • Recurrent swelling of the parotids
  • Presence of RF
  • Presence of purpura
  • Cryoglobulinemia
  • Splenomegaly, Lymphadenopathy
  • Low CD4+
  • Low C4
  • Presence of ectopic germinal centres
  • Germinal mutation TNFAIP3
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16
Q

Felty syndrome is associated with HLA DR4

A

True

Bone marrow examination shows normal myelopoiesis but with maturation arrest. There is increase susceptibility to infection as well as an increased risk of developing a lymphoproliferative disorder. Treatment involves rheumatoid arthritis treatment as well as granulocyte colony stimulating Factor to address the neutropenia.

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17
Q

What is the gender predominance for reactive arthritis?

A

M:F
3:1

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18
Q

Features consistent with secondary Raynaud’s

A

Male
Age >40
Abnormal capillaries in nailfold
Known precipitant
Ulceration
Asymmetric

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19
Q

RF for developing SRC in SSc

A

DIFFUSE SKIN INVOLVEMENT

OThers:
- steroid use
- cyclosporin
- presence of Ab

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20
Q

HLAb27 and inflmmatory conditions

A

Anks. Spond 90%
Reactive 70%
Psoriatic 50%
IBD 30%

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21
Q

Febuxostat; there is concerns re. increased mortality in which patietns?

A

IHD, CHF, hepatic failure

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22
Q

Large granular lymphocyte syndrome (LGL)

A

is a condition that is associated with rheumatoid arthritis. 1 and 3 patients with rheumatoid arthritis who have neutropenia have this condition. It is also associated with lymphocytosis, anaemia, and thrombocytopenia. Bone marrow assessment shows evidence of clonal lymphocyte proliferation. This condition is often managed with immunosuppressive therapy

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23
Q

NOD-like receptor protein 3 (NLRP3) inflammasome; which disease is it involved in?

A

gout

24
Q

Behcet’s

A

AU, genital ulcers and oral ulcers
HLA-B51

25
Q

Antibodies in antisynthatase sx

A

triad of interstitial lung disease, inflammatory arthritis, and inflammatory myositis
Antibodies against tRNA synthetase are present
Anti Jo1,
PL7
PL12

26
Q

Mixed connective tissue disease

A

female
30-40
Rayneud’s initial 90%

Anti U1RNP MUSTbe postive

Anti-U1RNP can be seen in other disorders such as systemic sclerosis and systemic lupus erythematosus, and so antibodies for these conditions, anti-centromere and anti-Scl70 along with anti-dsDNA, respectively, must be negative.

27
Q

FMF recessive or dominant

A

recessive

27
Q

FMF recessive or dominant

A

recessive

28
Q

for GCA, when does sensitivity of imaging drop after steriod tx?

A

2-4 days

29
Q

What is a biopsy requirement for the diagnosis of inclusion body myositis

A

biopsy must show in the endomysial inflammation

rimmed vacuoles with protein inclusion is not required

30
Q

what does HLA B27 suggest in reactive arthritis?

A

More severe and chronic disease

31
Q

IBD associated spondyloarthritis

A

1-20% of IBD patients
- more common in those with extraintestinal features, complications of bowel disease, large bowel in involvement in crohn’s

2 subtypes:
- axial - similar to AS
- peripheral:
usually acute, oligoarticular, LL
deformitis and erosions are rare
self limiting - related to flares of IBD

Tx:
NSAID
DMARDs if peripheral dx
Anti TNF for joints and bowel
Axial dx- treat as per AS (but avoid IL 17 blocker - secukinumab)

32
Q

what is the ANA pattern in dSSc

A

Nucleolar

33
Q

CCB are not effective in CTD related PAH

A

TRUE

even the usual treatments are less effective

interesting fact: iron deficiency correlated with worse prognosis in SSc-PAH

34
Q

SRC

A

Associated with RNA polymerase 3 ab
occurs within 5 years of diagnosis

Characterised by:
-abrupt onset of moderate to severe HTN
- normal urine sediment or only mild proteinuria with few cells or casts
- progressive renal failure
- does not cause glomerulonephritis
-
Occurs in upto 10-20% of patients with diffuse SSc and mucgh less frequently in those with limited SSc

RF: Tendon frictuon rub, RNA polymerase 3 ab

triggered by steroids

Signs of severe SRC:
- MAHA and thrombocytopenia
- HF and falsh pulm. oedema
- Blurred vision due to retinopathy
- HA, fever, malaise
- Encephalopathy +/- seizures
- Pericardial effusion

Treatment:
- if untreated can lead to ESRD within 1-2 months
- prompt BP control increases 1 year survival by 80%
OPTIONS:
- ACEi - captopril (first line)
- non first line: ARB, IV prostacyclin , ACEi and PLEX (better survival), ACEi and bosentan (prseved renal funcion)

CNS involvement: ACEi and IV nitroprusside

treatment with above even if normotensive

35
Q

High risk for SSc-SRC

A

dc SSc
older age
MALE
STEROIDS
RNAP3
ANA - speckeld
Pericardial effusion

36
Q

SSC-SRC

A

renal outcomes:
- 20-50% still progress to ESKD despite ACEi
- Dialysis is more difficult
- Improvment in RF can continue for upto 18mo
- Mortality is still high
- limited experience with renal transplant in SSc

HIGH BP at presentation = better outcome
if on ACEi prior to SRC = worse outcome
Temporary dialysis and no dialysis > permenant dialysis
MEN = poor prognosis

37
Q

SSC-Raynauds

A
  • avoid caffeine, stop smoking
  1. CCB (dihydropyridine)
  2. ARB, PDE5i, Alphablocker, SSRI
  3. IV prostacylin PGI2 in severe
38
Q

SSc-digital ulcers

A
  • avoid cold
  • pain relief
  • dressing
  • tx infection
  1. iloprost should be considered
  2. PDE5i considered
  3. Bosentan for reduction of number of new ulcers
39
Q

SSc - GI

A
  • Oropharyngeal: rigidity of facial musclces and tongue ==> swallowing difficulty
  • reduced saliva production
  • inco-ordination of swallow ~25%
    >oral leakage
    >retention
    >aspiration
    >difficulty swallowing

**GORD: 90% of pts with both diffuse and limited **
- absent motility in >50%
- Tx: soft food; avoid opioids, smoking, alcohol; PPT
- prokinetic (masopride)
- treat moniliasis (candidiasis)

40
Q

dSSc-GI

Small bowel

A

dysfunctional - bloating and distention
diverticulae
SIBO - diarrhoea, bloating, weight loss
Intussuception/volvulus
Pneumatosis intestinalis

41
Q

SSc

Management of abdominal pain/distention

A

Prokinetic:
Prucalopride
Domperidone/Metoclopramide
Erythromycin - powerful motilin agonist, causes powerful contraction

Oral procholinergic: pyridostigmine bethanacol

42
Q

Treatment options for SIBO

A

Doxycycline, metronidazole, ciprofloxacin
Rifaxamin

43
Q

Auto HSCT in SSc

A
  • conditioning regime is cyclophosphamide or rabbit ATG
  • Treatment related mortality was all within the first 60 days especially in smokers

disease relapse in 37%

Cardiac complication with HSCT:
- cyclophosphamide haemorrhagic pericarditis
- Radiation induced injury \

44
Q

SSc

SSc-cardiac

A

Microvascular CAD >60%
Primary RV dysfunction
LV diastolic dysfunction

45
Q

Which DM antibody is associated with higher risk of ILD including rapid progression and mortality?

A

Anti MDA-5 abs

46
Q

clinically amyopathic DM

A

10-30% of DM
Cutaneous features without muscle invovement
increased risk malignancy
More likely to be MDA5 +ve

47
Q

what are the cancer associated abs in DM>

A

Anti TIF1g
Anti NXP-2

48
Q

myositis specific abs predictive of good and bad prognosis

A

Worse: Jo-1, SRP
Better: Mi-2, overlap (RNP, PMScl, Ku)

49
Q

Treatment for DM,PM, IMNM

A

High dose GC, taper over year

Steroid sparing agents:
- AZA
- MTX
- IVIG
- RITUX
- PLEX

Monitor: CK, PFT, muscle power

50
Q

IBM

A

Asymm
Distal finger flexors
Dyspahgia
CK elevatd, but not useful for monitoring
15% have AI disease
EMG, MRI muscle biopsy
Less responsive to immunosuppresive therapy

51
Q

which joint does OA spare in the hand?

A

MTP joints

52
Q

hook osteophyte in the context of CPPD, indicates?

A

haemochromotosis

53
Q

Vasculitis

P-anca are associated with more frequent relapses

A
54
Q

Vasculitis

Hypersensitive vasculitis is also called:

A

cutaneous or leucocytoclastic

ARA criteria: 3/5
Age>16
Temporal relationship to drug
Palpable purpura
Maculopapular rash
Perivascular neutrophils on skin biopsy

Common triggers: frusemide, sulfonamides, penicillins, cephalosporins, allopurinols, phenytoin

55
Q

Vasculitis

MPA

A

Palpable purpura + systemic features
Glomerula haematuria, renal impairment
+/- abnormal LFTs
p-ANCA
50% of haematuria and haemoptysis is due to MPA or GPA
Mononeuritis multiplex

55
Q

Vasculitis

MPA

A

Palpable purpura + systemic features
Glomerula haematuria, renal impairment
+/- abnormal LFTs
p-ANCA
50% of haematuria and haemoptysis is due to MPA or GPA
Mononeuritis multiplex

RACP (20 decks)

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