RHEUM Flashcards

Question

Which part of the joint has no pain receptors?

Answer 1

Increased destruction - synovitis --> cytokines (IL1, TNF, IL6) down regulates aggrecan and collagen type 2 Increased deposition - BMP-2 and low TGF-beta --> osteophytes Cytokines trigger osteoclasts + osteoblasts Have CPPD at end stage

Answer 2

Meloxicam Celecoxib

Answer 3

Erosive 2nd and 3rd MCPs; does not improve with treatment

Answer 4

NF-kappa B Inflammasomes IL1 Interferons

Answer 5

itis Fevers Rash Lymphadenopathy esp arthralgias

Answer 6

Overproduction - Primary: PRPP hyperactivity, HGPRT1 def - G6PD, Fructose 1 ph aldolase def - Secondary: PV, hemolysis, TLSm hemoglobinopathies, Incr intake - fructose, alcohol, meat, seafood Under excretion - URATE1 GOF/ABCG2 LOF - Thiazides, loops - aspirin - pyrazinimide, cyclosporine

Answer 7

IL1 Inflammasomes TLR 2+4

Answer 8

CYP450 inhibitors - Cyclo - Clarithro

Answer 9

Aza Mercapto Thiazides: increases risk of AHS

Answer 10

HLA B5801 Han chinese, thai

Answer 11

Reduced secretion of cephs/ penicillins/ MTX - higher serum concentration

Answer 12

○ Hyper-rininemic, rapidly progressive renal impairment ○ Abrupt onset HTN ○ Mild proteinuria ○ MAHA Progressive renal failure

Answer 13

LIMITED - Skin involvement limited to upper limbs; gradual onset - A: perioral soft tissue loss - B: sclerodactyly ○ Tapered, cyanotic - C: facial teleangiectasia - D: dilated nailfold capillaries - E: calcinosis cutis - Clinically significant ILD more rare - pHTN + GERD and faecal incontinence similar DIFFUSE - Short history Raynaud's - Severe skin involvement --> contractures - MSK and internal organ comps --> ILD, renal crisis - Tendon friction rubs - Constitutional symptoms - Assoc: anti-Scl70, RNA polymerase III and I, ANA with nucleolar pattern - pHTN + GERD and faecal incontinence similar

Answer 14

Assoc with systemic sclerosis Gastric enteral vascular ectasia (GAVE) * Watermelon stomach * Stomach liniing thickened and fibrosed * Fe deficiency

Answer 15

- Systolic dysfunction: ACEI/ARB - Diastolic dysfunction: frusemide - Immunosupression for myocarditis

Answer 16

- MTX/ MMF - Low dose pred for friction rubs - Biologics: resistant arthritis * Hand, foot, nail care * Pruritis ○ Regular application of emollients + avoidance of soaps ○ Topical corticosteroids ○ Antihistamines * Hand/facial exercises

Answer 17

* BP control ○ ACEI/ARBs ○ Use additional if needed --> avoid beta blockers ○ PLEX if MAHA Dialysis

Answer 18

* Ulcers ○ Iloprost ○ PDE-5 inhibitors ○ Bosentan * Digital ischemia ○ IV prostaglandin/prostacyclin ○ Vasodilator therapy/antiplatelet/anticoagulation ○ Statin ○ Abx ○ Botox/ digital sympathectomy ○ Debridement for necrotic tissue * Raynauds ○ Avoid cold Botox

Answer 19

* ILD ○ Mycophenolate / mycophenolic acid ○ PO/IV cyclophosphamide ○ Other § Nintedanib § Pirfenidone § Ritux ○ Tocilizumab preserves FVC * PAH Continuous IV epoprostenol --> severe PAH

Answer 20

Non smokers non responsive for standard treatment - diffuse for first 4-5 years mild-moderate organ involvement - local with progressive organ involvement

Answer 21

- Reduced life expectancy * Mostly due to cardiopulmonary manifestations / pulmonary fibrosis/ paHTN - Renal crises * Higher BP = better outcome ACEI prior to crises = worse outcome

Answer 22

□ anterior ischaemic optic neuropathy (occlusion of posterior ciliary arteries) most common cause □ Posterior ischemic optic neuropathy □ Central retinal artery occlusion □ Choroidal ischemia Cerebral ischemia

Answer 23

Headache jaw claudication scalp tenderness

Answer 24

Visual symptoms - IV methylpred - Progression rare after initiating steroids - SE's ○ High risk infection in first year Tocilizumab - Refractory to steroids - IL-6 receptor inhibitor - Risk of relapse after being discontinued Aspirin - If other CVD risk factors MTX/ lefluonamide

Answer 25

o Lymphoma ○ Highest risk among all AI diseases ○ Predicting factors § Tongue atrophy § Persistent parotid gland enlargment § Purpura § Mixed II cryoglobulinemia § Low C4 § Autoantibodies § Extensive lymphocytic infilftration ○ Higher risk of NHL ○ DLBCL MALT

Answer 26

o Renal: RTA, cryos o Neuro o Fatiguability o Low grade fever o Raynauds o Myalgias/ arthralgias o Arthritis o AI epithelitis o Interstitial pneumonitis o Interstitial nephritis o RTA o Vasculitis

Answer 27

In individuals with suspected APS, warfarin is typically preferred over a direct oral anticoagulant (DOAC), although a DOAC may reasonably be used in selected individuals. Asymptomatic ○ Primary prevention contentious § Aspirin § HCQ Secondary prevention ○ Warfarin NOT DOACS LIFELONG aiming INR 2.5-3.5 Pregnancy ○ Prior thrombosis: therapeutic LMWH + aspirin ○ Prior pregnancy loss: low dose LMWH and aspirin daily

Answer 28

- Rare complication; high mortality rate Causes: sepsis, warfarin discontinuation, inflamm states Treatment: anticoagulation, PLEX, steroids

Answer 29

Lupus anticoag affected by warfarin; carries highest risk of thrombosis Beta 2 glycoprotein rare Anticardiolipin often positive DIAGNOSIS - Thrombosis/ pregnancy morbidity AND Antibody positive on 2/more occasions

Answer 30

§ Hydralazine § Isoniazid § TNF-alpha inhibitors: clinical lupus rare but can induce AA (ANA, antidsDNA, APL Abs) § Procainamide § Carbamazepine § Minocycline § Methyldopa § Chlorpromazine § Statins § Sulfasalazine § Cardiac: some ACEI/BB, statins, HCT § PTU § Antipsychotics: lithium, chlorpromazine § Anticonvulsants: carbamazepine, phenytoin

Answer 31

- Loss of self tolerance to auto antigens ○ Incr apoptotis material ○ Autoantigens form immune complexes with autoantibodies --> triggers inflammatory cascade ○ Self nucleic acids in complexes activate TLRs § Dendritic cells --> IFN § NETS --> TLRs § Elevated B cell survival factors BAFF/BLyS + APRIL - Major cells involved ○ Type I IFNs ○ B cells § End product: abnormal expansion of autoreactive B cells --> increased autoantibodies ○ Autoantibodies form immune complexes --> deposition + inflammation ○ T cells § Produce IL and proinflammatory cytokines - Lupus autoantigens accumulate inside cells: susceptible to modifications ○ Apoptotic debris accumulates --> AI response

Answer 32

ANA positive - Presnt in almost all patients - Some can be negative Sensitive not specific Anti-dsDNA >reference, if by ELISA 2x reference Specific for SLE --> can be negative if disease in remission Assoc: nephritis, TNFi induced SLE Homogenous pattern Anti-Smith MOST specific for SLE --> assoc with renal and CNS disease SSA + SSB 20-30% RO-52 associated U1RNP 25% Drug induced ANA more common antinuclear and antihistone antibodies and, in some cases, anti-double-stranded DNA (D-penicillamine, anti-TNF-α) or perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) Ribosomal P Abs/ antineuronal/ smith Neurolupus

Answer 33

Minor disease Hydroxychloroquine NSAIDs Steroids Major disease IV steroids MTX Azathioprine Lefluonomide If bad arthritis Mycophenolate - Renal lupus - ILD BIOLOGICS Belimumab B cell inhibition CD20 Tibulizumab Rituximab Anti CD20 depleting MAB Veltuzimab Leucocyte movements Anifrolumab IFN type 1 receptor antibody JAK INHIBITORS Baricitinib CAR-T CELLS, CD19 TARGETED

Answer 34

NSAIDs: avoid in T3 Steroids: low dose as possible AZA < 2mg/kg/day Hydroxychloroquine § May reduce congenital heart block CSA/tac

Answer 35

- Infectious/non infectious environmental agent --> inflammatory response involving accumulation of inflammatory cells - HLACD4 complex --> APCs present antigen to helper T cell --> IFN, TNF, IL12/18/8 - T cell mediated

Answer 36

pHTN Arrhythmias Heart block if AV node affected = MOST COMMON CARDIAC PRESENTATION BASAL SEPTAL LV ANEURYSM

Answer 37

* Maculopapular lesions * Hyper/hypopigmentation * Keloid * Subcutaneous nodules * erythema nodosum * skin: lupus pernio ○ Rash on face Papules on nose bridge/ area beneath eyes/ cheeks

Answer 38

Hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol) - Renal calculi

Answer 39

ACUTE - Steroids CHRONIC Indications: avoid end orgen/life threatening disease, improve QoL - Eye/heart/CNS Steroids first - Try and taper - If not tolerated/ ineffective consider other therapy Systemic therapy - Hydrozychloroquine ○ SE: ocular toxicity - Minocycline: cutaneous - Pulmonary/extrapulmonary: MTX, azathioprime, leflunomide, mycophenolate, cyclophosphamide Biologics - - etanercept - Golimumab ○ Not sig different to placebo - Infliximab ○ Improves lung function ○ Higher risk of of TB reactivation ○ SE's infection, carcinogen, allergic reaction