Rheumat notes Flashcards
What causes falsely high ESR?
NAPKIN
N = neoplasm
A = anemia, autoimmune
P = pregnancy
K = kidney disease
I = Infection, inflammation
N = neoplasm
Also remember
* Giant cell arteritis
* Macroglobulinemia
* Allergic vasculitis
* Hyperfibrinogenemia
* Necrotizing vasculitis
What is the serology for MSA (myositis specific antibodies)?
Why is it important to do serology?
Serology indicates the aggressive nature of disease and guides management
Anti-MDA5 is rapidly progressive and requires immediate high dose immunosuppressant
What are the anti-synthetase autoantibodies?
- Anti Jo-1
- Anti-PL-12
- Anti-PL-7
- Anti-EJ
- Anti-OJ
- Anti-KS
- Anti-Zo
- Anti-Ha/YRS
How to compare immune mediated necrotizing myopathy vs polymyositis?
Polmyositis: presence of lymphotic inflammation surrounding or invading myofibers. MHC1 is usually diffusely upregulated in polymyositis but negative in IMNM.
IMNM typically lacks visible visible lymphocytic inflammation. More necrosis and degeneration of the muscle fibers.
What needs to be tested prior to giving immunosuppression drugs?
- HBsAg, HBVDNA, anti HBc (worry about occult Hep B)
- Anti HCV RNA
- TB: IGRA (if positive ensure not active TB)
- HIV
If use biologics than start single drug anti TB 6 weeks prior to use of biologics
Isoniazid (must use with vit B6 to prevent peripheral neuropathy) given for 9 months
Rifampicin done for 4 months
What immunosuppressants are used in polymyositis/dermatomyositis etc
- Hydroxychloroquine for mild myositis
- Methotrexate
- Azathioprine
- MMF (mycophenolate mofetil)
- Calcineurin inhibitors: Cyclosporin A, tacrolimus
What is neonatal complication in pregnant mother with SLE?
What is treatment?
What is prognosis if treatment fails?
Heart block: can range from 1st degree to 3rd degree
Neonatologist must continously check fetus heart
Treatment: IM injection of steroids so can cross placenta and go to fetus –> suppress the inflammation and relieve heart block caused by Anti-Ro and Anti-La antibodies)
If cannot relieve the 3rd degree heart block fetus dies due to hydrops fetalis (no fetal movement: still birth)
If antiphospholipid syndrome is pregnant after previous miscarriage how to manage the patient?
How does management change if previous thrombotic event?
What is management if APS but no previous thrombotic events of pregnancy complications?
- Give oral low dose aspirin and subcutaneous injection of low molecular weight heparin (LMWH)
- Treatment is done until term not lifelong
- History of thrombotic vascular event: oral low dose aspirin and subcutaneous injections of therapeutic (high) doses of heparin
- Asymptomatic: patients with clinically significant aPL profiles –> no treatment or oral LDA
Continue warfarin after birth to prevent further thromboembolism formation. When pregnant must stop warfarin as teratogenic.
What are the 2 biologics that can be used for SLE?
Rituximab: anti CD20
Belimumab: anti BAFF monoclonal antibody
What immunosuppressants are acceptable at all stages of pregnancy
What are drugs that are acceptable in early pregnancy (but can be continued if required for disease control)
Avoid in all stages of pregnancy
What are the complications of Raynauds disease?
Classical triad?
Skin ulcers and rarely gangrene
Initially white/pallor from vasoconstriction, blue/cyanosis from sequestration of deoxygenated blood and finally redness from reperfusion and hyperemia.
Occurs with lupus (25%), sjogrens syndrome (33%) and nearly everyone with scleroderma.
* Eventually there is lumen constriction, coexistent edema of tunia intima and thickening of the tunica adventitia
How to test for small vessel vasculitis vs large vessel vasculitis?
Small vessel vasculitis: blood test serology for ANCA (EGPA, MPA (microscopic polyangiitis), GPA (granulomatosis with polyangiitis)
Large vessel vasculitis: do biopsy –> skin, nerve and kidney biopsy
How to classify vasculitis?
What is buergers disease (thromboangiitis obliterans) and how to manage?
Non atherosclerotic inflammatory disease of the peripheral blood vessels affecting small and medium sized vessels of extremities –> skin ulceration and gangrene
Shionoya clinical criteria
* Smoking history
* Onset before age <50 years
* Infrapopliteal arterial occlusions
* Either upper limb involvement or phlebitis migrans
* Absence of atherosclerotic risk factors other than smopking
Not autoimmune so dont give immunosuppressants
Treatment by vasodilator: prostaglandin analogues
Iloprost: prostacycline analogue
Trental: improves blood circulation
Verapamil
What antibodies tested in SLE?
- ANA: only for dx. Once positive no need to test again
- Anti-dsDNA: used for dx and monitoring for disease progression (esp for lupus nephritis)
Anti-ENA: dx and prognosis on development of complications
* Anti Sm: specific and associated with neurological involvement
* Anti-Ro/La: for primary sjogrens
* Anti RNP: a/w overlap features e.g suggestive of MCTD
* Anti centromere: limited systemic sclerosis
* Anti Scl70: diffuse systmeic sclerosis
* Anti Jo1: dermatomyositis (anti MLA5 –> rapidly progressive ILD)
C3,4: dx and monitoring of disease progression. Low levels in active disease –> check LFT to r/o decreased production in liver disease
What is the principle of Mx of SLE?
Induction and maintenance
HCQ for all levels (baseline fundoscopy done and annual F/U by opthal to detect complications): AE: corneal deposits, bulls eye maculopathy, blue grey skin pigmentation
Induction: short term high dose IV methylprednisolone for severe lupus
Maintenance: lower dose steroids+ non steroid agents (prefer MMF due to less AE)
Add methotrexate if refractory and persistent
Change DMARD into azathioprine if unreponsive to methotrexate after 3-6mo therapy
Biologics: Add belimumab (anti BAFF: B lymphocyte stimulator specific inhibitor)/ rituximab if patient still refractory
Rational of HCQ use in SLE at all stages?
(1) Stabilize ds and reduce flares
(2) Improve fetal outcome in pregnancy
(3) Improve outcomes for constitutional, cutaneous, musculoskeletal S/S
(4) Reduce CVS and infection risk
Cutaneous manifestation of SLE in ACLE, SCLE and CCLE?
Spectrum of connective tissue diseases
antibody markers of systemic sclerosis and its implication
Presentation of limited cutaneous systemic sclerosis vs diffuse systemic sclerosis?
Scleroderma renal crisis due to intrareal arterieal stenosis. ix: urinaanlysis, CBC and PBS: MIHA and thrombocytoepnia. Can lead to ESRD in 1-2 months. Low grade myositis
What is mixed connectiec tissue disease?
Specific generalized connective tissue disease under overlap syndrome that is a/w increased anti-U1 RNP
Features of SLE, SSc, PM
Ix: CBC: low grade anemia, leukopenia
AutoAb: ANA, anti-U1 RNP. RF and ACPA. antiendoethelial cell Ab
Diagnostic criteria for RA?
Mx?
NSAID for pain control
DMARD
Non biologics: sulfasalazine, methotrexate, HCQ, leflunoamide, gold
Biologics: anti TNF (adalimumab, infliximab, etanercept); anti IL6 like tocilizumab; anti CD20 like rituximab
What are common causes of thrombocytopenia?
RA vs psoriatic arthritis
RA: symmetrical, usually involving small joints of hands (PIP) and feet. Ulnar deviation, swan neck, boutonniere.
Psoriatic arthritis: asymmetrical, any joint can be affected (remember to check hair line). Dactylitis, enthesitis, bamboo spine (remember ABCDE)
