Rheumatology Flashcards
RF for hydralazine drug induced lupus
- high dose >200mg
- slow acetylator
- HLA-DRw4
- > 3month of therapy
- Female
- FHx of autoimmune
Antibodies associated with anti synthatase sx
Anti-Jo1
Anti PL-7
Anti PL 12
scleromalacia perforans
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
RA
Rheumatoid nodules are commonly seen in seropositive patients treated with Methotrexate alone.
Which clinical features of GCA is associated with positive biopsy?
Jaw claudication
GCA
Genetic association:
- IL17A, IL33, HLA DRB104
Which group of agents should we not precribe colchicine with due to increased risk of SE?
Strong CYP3A4 and P-gp inhibitors
amiodarone, cyclosporin, digoxin
diltiazem, antifungals such as itraconazole, macrolide antibiotics, statins and fibrates
What shoulder patholgy exhibits the painful arc?
Supraspinatus tendonitis
Secukinumab
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
What kind of ILD is RA more like?
UIP
Belimumab
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)
Histological features of GCA
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.
If you have GCA and your vision is threatened, what is the treatment?
Methylpred
Does the use of OCP increase or decrease the risk of RA?
Decrease (esp those who are CCP positive)
Factors that increase the risk of lymphoma in those with sjogren’s disease
- 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
Felty syndrome is associated with HLA DR4
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.
What is the gender predominance for reactive arthritis?
M:F
3:1
Features consistent with secondary Raynaud’s
Male
Age >40
Abnormal capillaries in nailfold
Known precipitant
Ulceration
Asymmetric
RF for developing SRC in SSc
DIFFUSE SKIN INVOLVEMENT
OThers:
- steroid use
- cyclosporin
- presence of Ab
HLAb27 and inflmmatory conditions
Anks. Spond 90%
Reactive 70%
Psoriatic 50%
IBD 30%
Febuxostat; there is concerns re. increased mortality in which patietns?
IHD, CHF, hepatic failure
Large granular lymphocyte syndrome (LGL)
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
NOD-like receptor protein 3 (NLRP3) inflammasome; which disease is it involved in?
gout
Behcet’s
AU, genital ulcers and oral ulcers
HLA-B51
Antibodies in antisynthatase sx
triad of interstitial lung disease, inflammatory arthritis, and inflammatory myositis
Antibodies against tRNA synthetase are present
Anti Jo1,
PL7
PL12
Mixed connective tissue disease
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.
FMF recessive or dominant
recessive
FMF recessive or dominant
recessive
for GCA, when does sensitivity of imaging drop after steriod tx?
2-4 days
What is a biopsy requirement for the diagnosis of inclusion body myositis
biopsy must show in the endomysial inflammation
rimmed vacuoles with protein inclusion is not required
what does HLA B27 suggest in reactive arthritis?
More severe and chronic disease
IBD associated spondyloarthritis
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)
what is the ANA pattern in dSSc
Nucleolar
CCB are not effective in CTD related PAH
TRUE
even the usual treatments are less effective
interesting fact: iron deficiency correlated with worse prognosis in SSc-PAH
SRC
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
High risk for SSc-SRC
dc SSc
older age
MALE
STEROIDS
RNAP3
ANA - speckeld
Pericardial effusion
SSC-SRC
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
SSC-Raynauds
- avoid caffeine, stop smoking
- CCB (dihydropyridine)
- ARB, PDE5i, Alphablocker, SSRI
- IV prostacylin PGI2 in severe
SSc-digital ulcers
- avoid cold
- pain relief
- dressing
- tx infection
- iloprost should be considered
- PDE5i considered
- Bosentan for reduction of number of new ulcers
SSc - GI
- 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)
dSSc-GI
Small bowel
dysfunctional - bloating and distention
diverticulae
SIBO - diarrhoea, bloating, weight loss
Intussuception/volvulus
Pneumatosis intestinalis
SSc
Management of abdominal pain/distention
Prokinetic:
Prucalopride
Domperidone/Metoclopramide
Erythromycin - powerful motilin agonist, causes powerful contraction
Oral procholinergic: pyridostigmine bethanacol
Treatment options for SIBO
Doxycycline, metronidazole, ciprofloxacin
Rifaxamin
Auto HSCT in SSc
- 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 \
SSc
SSc-cardiac
Microvascular CAD >60%
Primary RV dysfunction
LV diastolic dysfunction
Which DM antibody is associated with higher risk of ILD including rapid progression and mortality?
Anti MDA-5 abs
clinically amyopathic DM
10-30% of DM
Cutaneous features without muscle invovement
increased risk malignancy
More likely to be MDA5 +ve
what are the cancer associated abs in DM>
Anti TIF1g
Anti NXP-2
myositis specific abs predictive of good and bad prognosis
Worse: Jo-1, SRP
Better: Mi-2, overlap (RNP, PMScl, Ku)
Treatment for DM,PM, IMNM
High dose GC, taper over year
Steroid sparing agents:
- AZA
- MTX
- IVIG
- RITUX
- PLEX
Monitor: CK, PFT, muscle power
IBM
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
which joint does OA spare in the hand?
MTP joints
hook osteophyte in the context of CPPD, indicates?
haemochromotosis
Vasculitis
P-anca are associated with more frequent relapses
Vasculitis
Hypersensitive vasculitis is also called:
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
Vasculitis
MPA
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
Vasculitis
MPA
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
