Dunedin- Rheumatology Flashcards
What is the larger contributor for a risk of rheumatoid arthritis ?
(1) Smoking (2) genetics
What HLA is associated with RA?
HLA-DRB1
MHC Class 2, Chromosome 6
Describe some genes associated with RA
PTPN22 SNP- together with HLA-DRB1 gives 50% genetic risk
IL2RA (chromosome 10p15)
PTPN2 (Chromosome 18p11)
Chromosome 12q24 region
Describe Mucosal origins hypothesis
Describe Anti-CCP
As sensitive as, and more specific than, IgM RF in early and fully established RA
Marker of erosive disease
What other things can cause rheumatoid factor to be high?
Chronic viral hepatitis
infection
When diagnosing RA, what is the time frame?
> 6 weeks
Co-morbidities and RA treatment
*dose reduction MTX when eFFR <60mls/min
*Contraindication MTX, LEF liver disease or transaminitis
*Contraindication: Chronic infection
- Consideration: recent malignancy
*Manage actively: Hep B or Hep C
*Various specific CI- VTE and JAKI, CHF, and MS with TNFI
What is the first line treatment for RA?
Methotrexate with oral prednisone with weaning. if still active ADD cDMARDS, if still active ADD bDMARDS or tsDMARDS
(1) DMARDS (methotrexate, salazopyrine, hydoxychloroquine, leflunomide)
(2) Biologics (TNFalpha inhibitors, B-cell inhibition, Anti-IL-6 co stimulatory receptor blocker)
(3)targeted synthetic tsDMARDS (Janus kinase inhibitors)
glucocorticoids, NSAIDS
Any “cept” eg etanercept
What age group is at risk with JAK inhibitors?
> 65 years
Can immune checkpoint inhibitors be used in RA?
Yes
What is Peresolimab?
Peresolimab is a humanized IgG1 monoclonal antibody designed to stimulate the endogenous programmed cell death protein 1 (PD-1) inhibitory pathway.
can be used in mod-severe RA disease
Methotrexate and the lungs?
Methotrexate causes Acute PNEUMONITIS NOT ILD
What is the pathophysiology of scleroderma?
*Initial microvascular/endothelial damage
*Initial microvascular/endothelial damage, Followed by an autoimmune response with inflammation
*Finally characterized by diffuse fibrosis
What is the scleroderma spectrum
Limited cutaneous AKA CREST
Describe limited cutaneous systemic sclerosis vs diffuse cutaneous systemic sclerosis
Limited cutaneous: CREST
- calcinosis, Raynaud’s, oesophageal dysmotility, sclerydactyl, telangiectasia + PULMONARY ARTERY HTN
Diffuse cutaneous systemic sclerosis:
- extensive skin involvement: proximal limbs and trunk.
Renal disease
pulmonary interstitial fibrosis
What is the most common cause of death in diffuse cutaneous systemic sclerosis?
Pulmonary fibrosis
Discuss renal crisis in Diffuse sclerosis
*Precipitated by steroids
*Anti RNA Polymerase III
*abrupt onset of mod-severe hypertension
*urine sediment normal or reveals only mild proteinuria with few cells or casts
* progressive renal failure
Describe cardiac involvement in systemic sclerosis
*Ischaemia including in response to cold exposure
*contraction band fibrosis
*right ventricular changes with pulmonary HTN
Describe GI involvement in systemic sclerosis
*GAVE (watermelon stomach) with chronic blood loss
*oesophageal dysmotility and reflux
*‘shrinking stomach’ with early satiety
*small bowel dysmotility with bacterial overgrowth
*malabsorption
*volvulus
*Faecal incontinence
*cachexia
Describe auto-antibodies in scleroderma
Diffuse: Scl-70 (anti-topoisomerase), RNA polyermase III, U3-RNP
Limited: centromere, Th/To, U11/U12 RNP
Which antibody is associated with mixed connective tissue disease?
U1-RNP
What antibody is associated with Myositis/Scleroderma
PMScl 100/75
What is the relevance of absent antibodies in scleroderma?
increased association with malignancy
Describe nail fold capillaroscopy
What is the Rodnan skin score?
used in scleroderma, treatment is based on it
Treating scleroderma
Scleroderma renal crisis: ACEI
Scleroderma inflammatory arthritis: Methotrexate
Skin involvement: Methotrexate, MMF, IV cyclophosphamide, HSCT
Lung disease or cardiac disease: MMF
Pulmonary arterial HTN: PDE5i, ERA (bosentan, ambrisentan)
raynauds or digital ulcers: CCB
GI: high dose PPI, promotability agents, oesophageal dilation
Digitial ulcers and scleroderma
strong association with pulmonary HTN
Describe spectrum of vasculitis
Out of the ANCA vasculitis, which ones produce granulmonas?
GPA (wegeners)
eosinophilic granulomatous w polyangitis (EGPA)
Differentiate between PR3-ANCA and MPO- ANCA
GPA: PR3-ANCA (sometimes MPA too)
MPA: MPO- ANCA
What lung disease is pANCA associated with vs cANCA?
pANCA- UIP
cANCA- NSIP
Urinary eosinophilia
interstitial nephritis NOT vasculitis
Erythema nodosum
Livido reticularis