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1. Rheum RC > SLE > Flashcards

SLE Flashcards

1
Q

2019 EULAR/ACR classification

A

Only if ANA ≥1:80 and had ≥10pts

-Class 3/4 nephritis (10), class 2/5 (8), proteinuria ≥0.5g/d (4)

-Anti Sm/dsDNA (6)
-AIHA/thrombocytopenia (4), Leukopenia (3)
-Low C3 AND C4 (4), C3 or C4 (3)
-Any APLA AB (2)

-ACLE (6), SCLE/DLE (4), alopecia/ulcers (2)
-Arthritis (6)
-Pericarditis (6), effusion 5
-Seizures (5), Psychosis (3), delirium (2)
-Fever (2)

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2
Q

SLE risk factors

A

-DR2 and DR3 histocompatibility complex → present self Ag to self reactive T/B cells
-Complement deficiency: C1q, C2, C4 → less clearance of apoptotic debris and IFNa production
-Hormones: childbearing years, XXY, XO, DHEA as Tx

-Enviro: smoking, EBV, CMV, Silica, UV light, pesticides, demethylating drugs, gut microbiome

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3
Q

Acute cutaneous lupus manifestations

A

-Malar rash
-Bullous lupus
-Toxic epidermal necrolysis variant
-Maculopapular
-Photosensitive
-ulcers

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4
Q

Chronic cutaneous lupus manifestations

A

-Classic discoid, hypertrophic
-Lupus pannicullitis/profundus
-Mucosal
-Lupus tumidus
-Chilblains

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5
Q

Chilblain’s Tx

A

-Cold avoidance
-Smoking cessation
-Topical steroids
-Oral nifedipine

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6
Q

APLA positivity definition

A

+ LAC
-Medium/high titer ACA (IgG/M/A)
-Positive anti-B2-glycoprotein (IgG/M/A)

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7
Q

SCLE (subacute cutaneous)
vs DILE (drug induced)
vs DLE (discoid)

-Manifestations, antibody, prognosis

A

SCLE: skin only , SSA/SSB+ (can be ANA-), 5-10% –>SLE

DILE: systemic (no CNS/renal), Histone+, improve when DC drug

DLE: skin only, ANA-, rarely become SLE

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8
Q

ANA staining patterns: rim, speckled, nucleolar

A

-Rim = AB to deoxynucleoprot (most spec for SLE)
-Speckled = MC in SLE and other dz (least spec for SLE)
-Nucleolar = think scleroderma

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9
Q

Situations where ANA is negative

A

-Severe proteinuria (no Igs in serum to bind HEp-2 cells); ANA turns + after tx
-After cytotox therapy
-In dz remission

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10
Q

CNS SLE - diffuse vs focal differences

A

-Diffuse: transient, reversible w/ therapy, not assoc’d w/ pathologic abN

-Focal: acute in onset, PERMANENT w/ therapy, assoc’d w/ pathologic changes on autopsy

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11
Q

CNS SLE manifestations

A

Diffuse:
– Confusion, Coma
– MCI/Dementia
– Psychosis, A&D
– Headache, pseudotumor cerebri
– Aseptic meningitis

Focal:
– Stroke syndromes
– Seizures
– Chorea
– Ataxia, hemiballismus
– Demyelinating syndromes
– Transverse myelopathy

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12
Q

SLE CNS pathogenesis

A

Endothelial disfunction:
- SLE complement activation → microvasculopathy and BBB disruption → influx autoAB, cytokines → diffuse manifestations

-Procoagulant factors (APLA etc) activate endothelial cell → thrombosis/emboli → stroke/focal manifestations

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13
Q

SLE CNS manifestations autoantibodies

A

Diffuse:
– Antineuronal
– Ribosomal P → psych (esp psychosis)
– NMDA → cog dysfcn

Focal
– Anti-aquaporin 4/ neuromyelitis optica (NMO) → transverse myelitis +/- optic neuritis
– APLA → transverse myelitis, strokes

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14
Q

SLE pt w/ GTC seizure and hypertensity on MRI with normal LP

A

Posterior reversible encephalopathy syndrome

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15
Q

** Posterior reversible leukocencephalopathy risk factor **

A

-HTN
-Immunosuppression (esp CYCLOSPORINE)
-Autoimmune dz: SLE, PAN, Cryo, GPA
-Acute/chronic renal failure, TTP, HUS, sepsis, blood transfusion, contrast

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16
Q

PRES clinical manifestations and Ix and Tx

A

-HTN
-Seizures
-H/A, Confusion, ALOC, N/V, visual change, ARF

-MRI: vasogenic edema over cerebral hemispheres = increased T2 signals: cerebellum, brainstem, anterior cortex
-LP: normal, but can hv elevated protein

TX:
- Manage HTN w titratable agent (eg labetolol)

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17
Q

**PRES DDX **

A

-Diffuse NPSLE: seizure + increased T2 signals with LP that can be normal w/ elevated dsDNA, IL6, immune complex, B cell activation, and BAFF
-Aseptic meningitis
-CNS vasculitis
-APLA stroke
-Autoimmune encephalitis

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18
Q

SLE pt w/ bizarre behavior and delusions - DDX and Ix

A

-NPSLE
-Prednisone induce psychosis
-Causes of delirium

Ix:
- SLE labs,
- CNS AB (riosomal P, NMO),
- LP for CSF cell count/diff, prot/gluc, Cx, viral studies, IgG index, oligoclonal bands,
- Antineuronal AB,
- MRI brain/spine, EEG

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19
Q

Lung manifestations SLE

A

-Bilateral pleuritis
-Acute lupus pneumonitis +/- DAH
-ILD/fibrosis (usually in overlap SLE, but r/o drugs)
-pHTN (r/o CTEPH, OSA)
-Shrinking lung
-Cryptogenic organizing PNA (highly responsive to steroids; r/o antisynthetase)
-Infxn (r/o aspiration, atypical w/ tree/bud)

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20
Q

Shrinking lung pathogenesis

A

-Phrenic neuropathy
-Diaphragm myopathy
-Pleural fibrosis

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21
Q

Cardiac manifestations SLE

A

-Myocarditis (HF or tachy)
-Pericarditis (L sided pleural effusion if symptomatic)
-Coronary vasculitis (RARE)
-CAD and MI
-HTN (steroids or renal insuff)
-HCQ induced cardiomyopathy
-Valvulopathy (in APLA), Libman–Sacks usually on ventricular side of posterior MV leaflet or AV

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22
Q

SLE GI manifestations

A

-* Esophageal dysmotility (upper 1/3)
-* Pancreatitis
-* Serositis
-* Mesenteric vasculitis
-* Hepatitis
-* Intestinal pseudo-obstruction
-* Protein-losing enteropathy (positive stool fecal alpha-1 antitryspin or transferrin)

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23
Q

** Indication for renal biopsy **

A

-* Increasing Cr w/o another cause
-* Proteinuria ≥ 1 g/24 hours
-* Proteinuria ≥ 0.5 g/day plus hematuria or cellular casts

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24
Q

LN Classes

A

I/Minimal mesangial

-II/Mesangial proliferative:

III/Focal (A = active lesions C = chronic lesions)

IV/Diffuse (IV-S = segmental IV-G = global, A = active lesions C = chronic lesions):

V/Membranous:

VI/Advanced sclerosing:

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25
Q

LN Class 1 manifestations

A

None

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26
Q

LN Class 2 manifestations

A

Microscopic hematuria +/– proteinuria;

Rare HTN

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27
Q

LN Class 3 manifestations

A

Hematuria and proteinuria

+/– HTN, Decreased GFR, Nephrotic syndrome

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28
Q

LN Class 4 manifestations

A

-Hematuria, proteinuria (frequently nephrotic), cellular casts,
-Decreased GFR
-HTN,
-Hypocomplementemia
-Elevated dsDNA

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29
Q

LN Class 5 manifestations

A

Extensive proteinuria

-MINIMAL hematuria or renal function abnormalities

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30
Q

LN Class 6 manifestations

A

Chronic kidney disease

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31
Q

LN Pathology - features of activity

A

-Cellular proliferation
-Cellular crescents
-Fibrinoid necrosis
-Hyaline thrombi
-Intersititial inflammation
-Neutrophils
-Hypercellularity

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32
Q

LN Pathology - features of chronicity

A

-Glomerular SCLEROSIS
-Fibrous crescents
-Fibrous adhesions
-Interstitial fibrosis

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33
Q

Risk factors for LN –> ESRD

A

-* Black and Hispanic (especially males)
-* Lower SES
-* Poor med compliance
-* Comorbidities (DM, HTN)
-* Failure to normalize Cr or serum Cr of >2mg/dL on therapy
-* Failure to decrease proteinuria to <1 g/day within 6 mo of tx
-* Renal bx showing high disease activity (cellular crescents) & chronicity (interstitial fibrosis)

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34
Q

First line for LN
- Induction: Class 3/4, Class 5

A

Induction for Class 3/4
– IV Solumedrol 0.5-1g x3d then 1mg/kg prednisone (if crescents) or 0.5mg/kg (without crescents). Taper after few weeks
– MMF: 2-3g/d for 6 mo (MMF>CYC in blacks/Hispanic) OR CYC high dose (500-1000mg/m2 monthly x6mo) or low dose (Eurolupus 500mg IV q2wks x6 doses)
– Can switch btwn MMF and CYC if no effect, or to Ritux, CNI if no effect with either

-Class 5: pred 0.5mg/kg/d x6 mo PLUS MMF: 2-3g/d for 6 mo (CNI can be added to MMF, careful if renal insuff/HTN)

– Triple therapy: Belimumab + GC + MMF or reduced CYC if repeat renal flare or high risk kidney failure

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35
Q

First line for LN
-Adjunct therapies

A
  • HCQ (<5mg/kg/d)
  • ACE, ARB if proteinuria >0.5g/24h (BP target <130/80)
  • Statin if LDL >100mg/dL
  • Stop smoking
  • Counsel against pregnancy if active nephritis or Cr> 2mg/dL
  • Ritux controversial
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36
Q

Cyclo dosing
– Dose: high vs low
– Route: IV vs PO
– Resistant

A

High dose: Monthly 0.5–1.0 g/m2 IV × 6 months
-Low dose (less infxn/infertility): 500 mg IV q2wk × 6 doses

-Total exposure less with IV vs PO

-Recalcitrant –> Add Ritux 1g at day 1 and day 14

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37
Q

Cyclo Side effects

A

-Fertility,
-Bladder toxicity (hemorrhagic cystitis)
-Premature ovarian failure (>10-15g total dose and age >30)

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38
Q

Cyclo Protocol
– Prior
– Dose
– Monthly changes
– Adjuncts
– Post Infusion

A

A) Prior to CYC
– Premedication 15–30 minutes prior to CYC: dexamethasone 10 mg, lorazepam 1 mg, Zofran 8 mg
– Mesna (25% of CYC dose in milligrams) in 250 cc normal saline CYC infusion

-B) CYC 0.5–1.0 g/m2 of BSA mo x6 (0.5g/m2 if CrCl<35–40 cc/min; or if on IHD 0.4-0.5g/m2 8-10h before or after IHD)

-C) Adjusting monthly dose based on WBC 10-14d post CYC: reduce by 0.25g/m2 if nadir <3, or increase to 1g/m2 if nadir >4

-D) Consider GnRH (Lupron) 3.75 mg IM 10d pre-CYC dose or testosterone (200 mg IM every 2 weeks) for men to prevent premature gonadal failure from longstanding therapy

-E) Post CYC infusion
– Mesna (25% of CYC dose in mg)
– Compazine SR 15mg BID prn or compazine 10 mg TID prn for 2–3 days

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39
Q

LN Maintenance therapy
– Duration
– Options
-

A

> 1-2years post induction

-* AZA (up to 2 mg/kg/day) (or 6-mercaptopurine if nausea on AZA) or MMF generally preferred (1–3 g/day)

-* No AZA if on allopurinol or warfarin (warfarin resistance)

-* CYC IV q3mo s/p induction if can’t tolerate AZA/MMF.

-* Prednisone is tapered over time to dose that controls renal and extrarenal manifestations

-Other:
-* Rituximab: limited data
-* CNI (cyclosporine, tacro): alone or combined with low-dose MMF

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40
Q

LN recommendations in pregnancy

A

Early preg: Therapeutic abortion or early immunosuppression to avoid teratogenicity

-Late: HCQ, Pulse steroid, AZA, Tacro
CYC if life threatening

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41
Q

Tx for Young SLE with proteinuria 2g/day and Bx with class 3/ 4

A

HCQ. Induction with MP and MMF (3 g po daily) followed by MMF (2 g po daily) maintenance for at least 3 years.

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42
Q

**SLE with casts, Cr 300 and Bx with 3 /4 **

A

HCQ. MP + IV CYC or MMF

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43
Q

SLE with class 3/ 4 but pregnant 4 months

A
  • HCQ
  • IV MP (ideally nonfluorinated GC eg betamethasone or dex) + TAC for induction

If severe, consider RTX.
Followed by either TAC or AZA for maintenance.

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44
Q

** SLE with membranous nephritis and proteinuria 2.5g Tx **

A

HCQ. ACEi or ARB for proteinuria.

-GC + MMF / AZA / CYC / CNI / RItux based on extrarenal mx or if nephrotic syndrome (>3g)

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45
Q

Trial for RItux in LN

A

LUNAR - no difference btwn Ritux and Placebo for renal response but may hv role in MMF incomplete responder

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46
Q

Trial for MMF vs AZA

A

ALMS and MAINTAIN
-MMF> AZA

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47
Q

Heme manifestations in SLE

A

-* Cytopenias (AIHA, AoCD, leuk/lymph/neutro-, thrombocytopenia)
-* APLAS
-* TTP
-* MAS (R/O EBV or CMV infection as a trigger)

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48
Q

MAS clinical/lab manifestations

A

-Fever,
-Splenomegaly,
-Organ dysfunction,

-Cytopenias,
-Low ESR - liver inability to synthesize proteins such as fibrinogen,

-High TGs, ferritin, IL-2 receptor [CD25])

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49
Q

Hemolytic anemia W/U

A

-LDH, bili, hapto, CBC, smear, retic count
-DAT

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50
Q

Thrombocytopenia Tx
– When
– How

A

Tx if Plt <30 or bleeding with:
– GC (Dex/Pred or pulse if severe)
– IVIG 2g/kg (400mg/kg/d x5d) in prep for splenectomy or if bleeding
– Splenectomy (NOT 1st line → clot/sepsis)
– Ritux
– Thrombopoeitin R Ag (romiplostin, eltrombopag, avatrombopag) if failed GC
– For Rh+ nonsplenectomized: anti-D (RhD)

-2nd line: AZA, MMF, Cyclosporine, tacro, Danazol (androgen), CYC, fostamatinib, dapsone, vincristine

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51
Q

TTP clinical and lab manifestations

A

-Fever,
-Anemia (microangiopathic hemolytic),
-Thrombocytopenia,
-Renal,
-CNS

-SCHISTOCYTES (vs spherocytes in Coombs + AIHA)

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52
Q

TTP PathoPhys

A

AB against ADAMTS13 → vwF NOT cleaved → large multimers binding glycoprot R → plt adhesion and microthrombi

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53
Q

TTP Tx
– 1st line
– 2nd line
– Refractory

A

PLEX followed by FFP (replace ADAMTS13)

-Less effective: antiplatelets, GC, immunosuppressive

-Refractory: Eculizumab (Monoclonal AB to C5)

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54
Q

SLE MSK manifestations

A

Tenosynovitis

-Nonerosive arthropathy (Jaccoud)

-Deformities (reversible but fixed late) 2/2 lax joint capsule, tendons,ligaments → MCP subluxation, ulnar deviation, swan neck

-Rhupus: erosive symmetric polyarthritis (RF, CCP +)

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55
Q

TNFi for IA in SLE - why and why not

A

Maybe for erosive IA (rhupus)

Why not:
– Concern for DILE

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56
Q

how does TNFi cause lupus

A

TNFi suppress Th1 cytokines driving immune response towards Th2 cytokine production, IL10 and IFN alpha

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57
Q

SLE Tx w/o organ involvement

A

-HCQ
-MTX, LFN, AZA, MMF

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58
Q

NSAID caution in SLE - which and why

A

-COX2 specific inhibitors: increase thrombotic risk in APLA+
-Celecoxib is sulfa based → rash in pt w/ SLE
-Ibuprofen can cause aseptic meningitis

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59
Q

SLE rash pathology and immunofixation findings

A

-Interface dermatitis

-Biopsy shows Igs deposited at dermal epidermal junction on IF

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60
Q

MC Mucocutaneous SLE lesions and description

A

MC:
– Oral ulcers - painless and on hard palate
– Malar rash - erythematous plaques sparing nasolabial folds
– Discoid - hyperkeratotic rash w/ mucus plugging, causing scarring alopecia
– SCLE - annular (red, raised, irregular edges, central clearing, nonscarring (sun exposed area)) vs papulosquamous (eczema/psoriasis)

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61
Q

Less common SLE rashes

A
  • Bullous lesions
    – Purpura
    – Urticaria (SVV - test anti c1q)
    – Panniculitis w/ subcutaneous nodules (lupus profundus)
    – Livedo reticularis (in APLA)
    – Perniosis (distal vasculopathy)
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62
Q

SLE mucocutaneous Tx

A

Nonpharm: photoprotection

-Pharm:
– HCQ (or chloroquine),
– Dapsone (if vesicular),
– Thalidomide (severe mouth ulcerations; must be on OCP)
– Belimumab (recalcitrant discoid/subacute rash)
– MTX, MMF, AZA, Ritux

63
Q

Discoid lesion Tx

A

-HCQ
-Topical CNI
-Corticosteroid: topical, oral, intralesional
-Belimumab if recalcitrant

64
Q

SLE alopecia causes

A

-Active disease → diffuse stress alopecia (reversible)
-CYC → diffuse (reversible)

-DLE → patchy in distribution of discoid lesions (permanent bc follicles damaged)
-Vasculitis/AB against follicles → focal patchy alopecia areata like presentation
-

65
Q

Skin lesion topical steroid therapies

A

Face = low/medium potency NONfluorinated (eg hydrocort)

-Trunk/arm: medium FLUorinated (eg betamethasone valerate, traimciniolone)

-Hypertrophic: high FLUorinated (betamethasone, clobetasol)

-Topical tacro (alternative to topical GC)

66
Q

SCLE Tx:

A

Non pharm: Stop smoking (impairs HCQ)

-HCQ (best for tumid LE>SCLE>DLE)
-GC <30mg/d
-Belimumab (takes 4-6mo)

67
Q

SLE Tx for
– Bullous
– SCLE
– DLE
– Lupus profundus
– Chronic lesions >50% body
– Vasculitis
– Hyperkeratotic lesions

A

– Bullous lesions: dapsone (measure G6PD first)
– SCLE: MMF, retinoids, CNI, dapsone
– DLE: HCQ+/- quinacrine, thalidomide (beware neuropathy), cyclosporine. Ritux
– Lupus profundus: Dapsone
– Vasculitis: immunosuppressives
– Hyperkeratotic lesions: oral retinoids

68
Q

SLE Indications for high dose GC (>1mg/kg/d)

A

-CNS lupus (eg transverse myelitis)
-Pneumonitis, DAH
-Serious complications from pleuritis, pericarditis, peritonitis
-Severe nephritis
-Vasculitis w/ visceral organ involvement
-MAS
-Thrombocytopenia w/ Plt <30
-AIHA

69
Q

**AVN risk factors **

A
  • CYC, GC use (eg pred 20mg/d >30d)
  • Vasculopathy: APLA AB, Raynaud’s
  • Disease activity: CNS, renal, cytopenias
  • Comorbidities: HTN, DM
  • Smoking
70
Q

AVN Tx

A

Core decompression if >25% femoral head involved w/o bony collapse

71
Q

Nonpharm Tx of SLE

A

Photoprotection: sunscreen (SPF 30+), avoid smoking (affects HCQ), camouflage cosmetics

-Avoid triggers: sun, sulfa abx, high estrogen OCP, alfalfa sprouts, echinacea

-Prevent atherosclerosis: control BP (target <130/80), LDL<100, no smoking, treat high homocysteine lvls

-OP prevention: Ca 1000mg, Vit D 800, minimize GC, bisphosphonate if >20mg pred daily for 3+ mo (controversial for premenopausal)

-Immunizations: HPV<26yo, flu, shingrix, hep b, pneumococcal
-*NO live attenuated if pred 20/d or DMARDs (MMR, polio, HSV, smallpox, yellow fever)

-Prevent infxn: IE ppx if APLA+mumur, TBST or quantiferon if pred >15/d, PJP if CYC or GC >15-20mg/d

-Prevent renal progression in LN: avoid NSAID, BP target 130/80, ACEi/ARB to decrease proteinuria by 30%

-Prevent clots in APLA: ASA if LAC+, HCQ, avoid unnecessary surgery/catheters, tx infxns, avoid COX2i, avoid exogenous E2 (OCP, HRT, SERM)

-Tx fatigue: R/O hypothyroid metabolic, A&D, OSA, meds. Can tx w/ HCQ, modafinil, DHEA

-Cancer screen

-Birth control: low/medium exogenous E2 OCP ok if mildmod SLE w/o clot risk (APLA, nephrotic syndrome, severe renal dz, clot hx, migraines), if clot risk → Prog IUD/OCP

-Screen for low vit D (target >30-40ng/mL) - r/o celiac

72
Q

**Conditions w/ increased incidence in SLE but not directly related to SLE **

A

-Cardiovascular disease
-Anxiety and depression
-Steroids related issues: osteoporosis, AVN, metabolic syndrome (HTN, obesity, DM)

73
Q

SLE Tx approach
– Mild (list symptoms)

A

–Mild (fatigue, arthritis, rash, serositis)
– NSAID (**worsens Cr, photosensitivity, aseptic meningitis)
– HCQ
– Low dose pred <20mg/d
– MTX or LFN / AZA

74
Q

SLE Tx approach
– Mod
-

A

–Mod (minor unresponsive esp low C3/4, and high dsDNA)
– Same as low: HCQ, GC (20-40), MTX/AZA
– MMF, AZA,
– Belimumab
– CNI

75
Q

**Evidence for Belimumab **

A

BLISS showed better response when combined w/ standard therapy (MMF or CYC-AZA) vs standard therapy alone in AUTOANTIBODY POSITIVE SLE

  • more renal response (UPCR <0.7, GFR no worse than <20% preflare, no rescue therapy), less renal related death

-Post hoc analysis howed best in MSK and mucocutaneous domains (less worsening in Heme, immunological, renal)

76
Q

Belimumab in pregnancy

A

-Discontinue at conception
-Discontinue during pregnancy
-Minimal transfer during breastfeeding

77
Q

MoA anifrolumab

A

IgG1-kappa monoclonal AB blocks Type 1 interferon R preventing inflamm/immunological response from IFN a,b,e, k, omega

78
Q

SLE Tx approach
– Severe

A

Severe (nephritis, CNS, pneumonitis, vasculitis, severe cytopenia
– Pulse steroids then high dose PO
– Induction: CYC or MMF
– Maintenance: AZA, MMF, CNI, combo
– Ritux
– Additional: PLEX, IVIG (autoimmune cytopenias, APLAS), stem cell transplant (refractory)

79
Q

SLE Tx target

A

Remission
– SLEDAI = 0
– HCQ
– No GC

-Or

-Low disease activity:
– SLEDAI <4
– HCQ
– Pred <7.5
– Immunosuppressives

80
Q

When to use PLEX in SLE

A

-DAH,
-TTP,
-Anti-NMO,
-APLAS

81
Q

Reasons for high CRP in SLE

A

-Vasculitis
-Serositis
-Infection

82
Q

Why is ESR always high in SLE even if dz quiescent

A

Persistent hypergammaglibulinemia

83
Q

Signs of infection vs SLE

A

-Complement rises
-WBC “normalizes” from usual low; LEFT shift
-Elevated procalcitonin or lactate

84
Q

How does stem cell transplantation work in SLE

A

Eliminates autoreactive lymphocytes and replaces them w/ undifferentiated cells

85
Q

MC cause of death SLE

A

Infxn: bacterial, fungal, TB, nonTB, mycobacterial, viral

-Active disease: LN, CNS, vasculitis, pneumonitis

-Cardiovascular: CAD, stroke, PAD

-Cancer: HPV (from poor viral clearance), NHL, Lung Ca, SCC (from discoid)

86
Q

MC Biologics in SLE, MoA and indication

A

Belimumab - monoclonal AB inhib BAFF/BLyS to prevent B cell survival. (BAFF/BLyS normally promotes B cell survival and prevents apoptosis)
- Indication: MSK or mucocutaneous manifestations (renal, CNS excluded from trials)

-RItux - antiCD20 monoclonal AB
– For recalcitrant dz, CAPS, or combined w/ CYC for B cell depletion

87
Q

Factors less likely to respond to belimumab

A

-Cytopenias
-Serologically inactive

88
Q

Black box warning for belimumab

A

Depression

89
Q

Belimumab dose

A

-10mg/kg IV monthly
-200mg SC weekly

90
Q

Drug induced lupus erythematosus classification

A

-Systemic DILE: arthralgia, myalgia, serositis, constitutional sx s/p 1mo of drug . +ANA and one other SLE critieria

-Drug induced SCLE - similar to idiopathic SCLE: photosensitivity, cutaneous lesions (vasculitic/bullous) **suspect if SCLE >50yo

-Chronic cutaneous DILE - discoid lesion s/p fluorouracil

91
Q

** Systemic DILE Drugs (high, mod, low risk) **

A

High: Procainamide, Hydralazine

-Mod: Quinidine, Penicillamine

-Low: Isoniazid, Methyldopa, Minocycline, AntiTNFalpha, IFN alpha, chlorpromazine

-Possible: anticonvulsants, PTU, SSZ, Li, HCTZ, amio, anti-PD-L1 immunotherapy , statins

92
Q

Drugs causing SCLE

A

-HCTZ, CCB, ACEi
-Statin
-AntiTNFa
-Leflunomide
-PPI
-Bupropion
-Docetaxel, Hydroxyurea, anti PDL1

93
Q

Systemic DILE manifestations

A

-SLE sx (Fever,arthritis/arthralgia, myalgia, serositis)
-Hepatomegaly
-Derm: Erythematous papular rashes (discoid and malar UNCOMMON)

-RARE: severe manifestations eg cytopenias, renal, CNS,

-dsDNA, low C3/4 ONLY if TNFi and interferon alpha

94
Q

DILE pathogenesis

A

-Genetics: slow acetylator phenotype

-Epigenetic: methylation changes → certain peptides overexpressed (eg LFA1) → autoreactivity

-Activated neutrophils → ROS release + oxidation → cytotoxicity

-NETs → autoantigen exposure stimulate autoreactive T/B cells

95
Q

Procainamide vs hydralazine induced lupus

A

-Procainamide: pleuritis +/- pericarditis; antihistone H2A-H2B-DNA complex

-Hydralazine: rashes; antihistone H3/4 complex

-Both: arthralgia/arthritis, myalgia, fever

96
Q

Minocycline induced lupus serologies

A

-Anticardiolipin AB
-pANCA
-Antihistone

97
Q

IFNalpha DILE manifestations and Tx

A

Typical SLE:
– oral ulcers
– alopecia
– nephritis

-Require GC and SLE Tx

98
Q

TNFi DILE manifestations

A

-Arthralgias,
-Rash
-Heme: leukopenia, thrombocytopenia
-** dsDNA AB **
-Hypocomplementemia

-Less likely antihistone

99
Q

Antibodies seen in DILE

A

-ANA (more common than symptoms)

-Antihistone (not specific to DILE; rare in TNFi) - IgG

-dsDNA (** only w/ anti-TNFa or IFNa **)

-APLA (with procainamide, quinidine, chlorpromazine) - rarely assoc’d w/ clots ; IgM

100
Q

Drug induced SCLE vs idiopathic SCLE

A

-Cutaneous SCLE more widespread, bullous or vasculitic
-SSA+ disappears when drug stopped

101
Q

DILE Tx

A

-Stop Drug
-NSAID for arhtralgia
-GC for severe pericarditis/pleuritis
-HCQ if prolonged

-AZA/CYC ONLY if drug induced vasculitis

102
Q

MCTD vs Overlap vs UCTD

A

MCTD: RNP+ w/ manifestations seen in SLE, SSc, and IIM

-Overlap: features of >1/6 SARDs (SLE, SSc, PM, DM, RA, SS)

-UCTD: some features of 1+ SARD w/ +ANA but doesnt meet criteria for any

103
Q

**MC MCTD manifestations **

A

-Raynaud
-Swollen hands w/ puffy fingers
-Synovitis (Jaccoud’s, erosions)
-Myositis
-Acrosclerosis
-LACK of renal/CNS

-Fever
-Lymphadenopathy
-Serositis

-RARE: myocarditis, HTN crisis, aseptic meningitis

104
Q

MCTD Derm manifestations

A

-Scleroderma,
-Skin rash,
-Oral ulcers

105
Q

MCTD GI manifestations

A

-Esophageal dysmotility
-Esophageal sphincter hypotension
-GERD
-HSM
-Hepatitis, pancreatitis
-Intestinal vasculitis

106
Q

MCTD Pulm manifestations and Ix (imaging, PFT)

A

-PAH (highest risk if nailfold abN)
-Pleuritis / Effusions
-ILD - NSIP

-PFT: restrictive, DECREASED DLCO, pHTN

107
Q

MCTD mortality cause

A

pHTN

108
Q

MCTD lab findings

A

-Lupus labs (anemia, Leuko/lymphopenia)
-ANA, RNP, hypocomplements
-RF
-Hypergammaglobulinemia → high ESR

109
Q

MCTD CNS manifestations

A

-Trigem neuralgia
-Sensorineural hearing loss
-Headache
-aseptic meningitis,
-seizure,
-peripheral neuritis,
-cerebrovascular disease
- psychosis

110
Q

Role of RNP in pathogenesis of MCTD

A

Molecular mimicry:
-HLA-DR4 mounts response against CMV glycoprotein that cross reacts with RNP 70kD polypeptide modified during apoptosis

111
Q

MC dz in Overlap syndrome * what dz are assoc’d

A

Sjogren’s = MC in overlap

-Seen with RA, SLE, SSc, PM, MCTD, PBC, necrotizing vasculitis, autoimmune thyroiditis, chronic active hepatitis, mixed cryoglobulinemia, hypergammaglobulinemic purpura

112
Q

Other overlap syndromes

A

-SLE w/ IIM
-SLE w/ RA
-SSc w/ IIM
-Limited SSc w/ PBC
-SSc w/ AAV
-Myositis overlap syndromes (antisynthetase)
-RA w/ SSc, SLE, MCTD, SS

113
Q

UCTD clinical manifestations

A

(SLE without organ involvement)

-Arthralgia/arthritis
-Raynaud’s
-photosensitivity,
-oral ulcers
-SICCA
-Serositis

-RARE Maj organ involvement

114
Q

UCTD lab manifestations

A

-ANA+
-Occasional SSA or RNP

-Usually no Sm, dsDNA, centromere

115
Q

UCTD classification criteria

A

-Signs/symptoms of CTD ≥3y but not fulfilling any criteria
-ANA+ on 2 occasions

116
Q

** Raynaud primary vs secondary**

A

Primary:
- Young F>M,
- ANA negative,
- NORMAL nailfold capillaries
- No peripheral vascular disease,
- No digital ischemia/ulcers/pitting/fissuring/gangrene,

Secondary:
-Older (>40), M, Asymmetric,
- ANA+ (or ENA/SSc ABs)
- ABNORMAL nailfold capillaries
- Ischemia proximal to fingers/toes, ulcers, pits, gangrene
- Other CTD findings (rash, sclerodactyly)

117
Q

Raynaud labs to send to work up

A

-Cryo
-Hypercoagulable workup
-TSH (for hyPOthyroid)
-SSc AB

118
Q

Raynaud reason for imaging

A

-Asymmetric
-Suspicion for thromboembolism, Buerger’s
-Abnormal Allen’s test
-Severe disease resistant to Tx

119
Q

Raynaud imaging options

A

-Brachial finger index measurement (>20mmHg gradient suggest prox fixed obstruction)
-Doppler
-Finger photoplethysmography
-Invasive angio or MRA for prox vessel dz (eg vascular thoracic outlet syndrome)

120
Q

Primary Raynaud pathophys

A

-Increased basal sympathetic adrenergic tone and increased alpha2 adrenoR activity at neutral temp → heightened fx in cold

-Nutritional bloodflow diminished but preserved = NO ulcerations

121
Q

Secondary Raynaud pathophys

A

-Reflex cold induced sympathetic constrictor nerve activity
-Dysfunctional endothelium: releases endothelin (constrict) and reduced vasodilators (NO, prostacyclin)

-SSc: intimal prolif and abN PLT adhesion → reduced lumen size & less flow through distal capillary loops → ulcers

122
Q

Raynaud’s nonpharmTx

A

Nonpharm:
-Avoid cold,stimulants (decongestant, amphetamines, diet drugs),BB
-Stop smoking,
-Stress management,
-Core warming, Mittens

123
Q

Raynaud’s Pharm Tx

A

Mild/mod:
– DHP-CCB (nifedipine XL 30mg/d, amlodipine 5mgd),
– NonDHP: Dilt 120mg/d
– Topical nitrate (¼-½ inch BID/TID, rest for 12hr to prevent refractory)
– ARB (losartan),
– PDE5i (sildenafil or tadalafil),
– SSRI (fluoxetine)

Severe or ulcers:
– Combo therapy w/ PDEi or ASA (81mg)

Recurrent severe/ulcers:
– Add prostaglandin (epoprostenol or iloprost) or botox or both.
– Start endothelin-1 inhibitor (bosentan) for scleroderma w/ recurrent digital ulcers

124
Q

Raynaud meds - which reduce new ulcer formation

A

Endothelin -1 ANT
-Statins (inhib rho kinase pathway that regulates alpha2 adrenoR expression)

125
Q

RP Drugs and their side fx / contraindication

A

CCB: edema, constipation, presyncope, H/A, GERD; AVOID in preg; use DHP if PH or LV dysfcn

-Symphatolytic (eg prazocin): postural hypotension

-Topical nitrate: H/A; use alternative if HF or PH, dont’ use with PDE5i

-Endothelin ANT (bosentan): LFT abN, H/A, flushing, edema

-Prostacyclin analogue: flushing, jaw pain, H/A, diarrhea, nausea

126
Q

Raynaud Tx for refractory

A

-Gangrene/amputation
-Digital sympathectomy
-Chemical sympathectomy via digital lidocaine block for pain

127
Q

** Causes of Raynaud**

A

Systemic: SLE, MCTD, IIM, SS, RA, Burger, Vasculitis, pHTN,

-Trauma: rock driller, lumberjack, CTS, hammer operator, frostbite, hypothenar hammer syndrome

-Drugs/Chemical: BB, cocaine, amphetamine, methylphenidate, IFNa, cisplatin, bleomycin, ergot

-Occlusive artery disease: postembolic/thrombotic arterial occlusion, thoracic outlet syndrome

-Hyperviscosity syndrome: polycythemia, cryo, paraproteinemia, thrombocytosis, leukemia, cold

-Endocrine disorders: carcinoid, pheo, hypothyroid

-Infection: IE, lyme, mono, hepatitis

Misc: CRPS, peripheral AV fistula,

-Cancer: ovarian, angiocentric lymphoma, paraneoplastic, hypothyroid

128
Q

Why triphasic RP

A

Vasospasm → pallor (most definitive)
-Static venous blood deoxygenates → cyanosis
-Rewarming → rubor

129
Q

Raynaud’s precipitant

A

-Cold (esp when accompanied by pressure)
-Sympathetic stimulation (pain, emotional distress, meds)
-Trauma
-Hormones (eg estrogen)
-Medication
-Smoking

130
Q

Sites of vasospasm in RP

A

Primary: acral (finger, toes, ear, nose, nipple)

Secondary: acral but also coronary arteries and vasculature supplying internal organs

131
Q

Give 3 Names for B cell receptors and their function

A

BLyS receptor 3 (BAFF-R) –> induces survival and activation of B cells via NFkB

Transmembrane activator and calcium modulator and cyclophylin ligand interactor (TACI) –> inhibits B cell expansion and enables class switching

B cell maturation antigen (BCMA) –> plasma cell survival

132
Q

Contraindications or indications that are not approved for Belimumab

A
  • Anaphylaxis
  • Ongoing infxn
  • Live vaccine w/i 30d
  • Preg
  • Avoid use w/ Ritux or CYC

Not approved:
- Severe active LN or NPSLE
- Not approved for RA

133
Q

2 methods to measure antidsDNA
- Difference in results
- How to increase specificity

A

Farr assay - radiolabelled DNA incubated w/ serum. Ammonium sulphate precipitates out immune complexes. Limited to high affinity AB, and is SPECIFIC for SLE but not highest sensitivity. GOLD STD

ELISA - DNA on solid phase support exposed to serum amd antiDNA AB binding detected w/ enzyme labelled AB to human Ig. Higher sensitivity than Farr assay.

Crithidia luciliae assay - circular dsDNA of parasite exposed to antibody w/ increasingly dilute concentrations until no more reactivity. Circular DNA less likely denatured = more specific than ELISA but less sensitive

134
Q

Diseases assoc’d w/ positive ANA

A

Rheum: RA, SS, SSc, MCTD, PM/DM, SLE, DIL

Infxn: HIV, HCV, IE, syphilis, EBV, Parvo, TB, Mono

Autoimmune: Hashimoto, Grave, Autoimmune hepatitis, PBC, IBD, UPF

Cancer: lymphoprolif, paraneoplastic

135
Q

Inteferon in SLE:
Role? MC family? Cell type associated?
What is an interferon signature?

A

MC: Type 1 IFN
Associated cell type: plasmacytoid dendritic cells
IFN signature: pattern of increased IFN gene expression (type 1 signature in SLE)

136
Q

Stimulating factors for IFN1

A
  • Inteferogenic immune complexes: autoantibodies + nucleic acid binding proteins via;
  • NETs: not degraded in SLE bc reduced extracellular DNase 1 –> more exposure of nucleic acid/prot to autoAB and autoreactive B cells
  • UV light: induce ROS to cause DNA damage –> recognized by autoantibodies to form immune complex to induce pDC production of T1 IFN.
137
Q

Role of NETs in SLE?

A

NETs: cell death pathway where neuts extrude nuclear material (histones, chromatin, cytoplasmic prot) in web structure.
Increased NET in SLE bc not degraded due to reduced extracellular DNase 1 –> more exposure of nucleic acid/prot to autoAB and autoreactive B cells

138
Q

Reasons to choose MMF over CYC

A
  • Better outcomes in African american and Hispanic
  • No fx on fertility, risk of hemorrhagic cystitis/bladder cancer
  • Less risk of infection
  • Convenience, taken PO
  • Can be taken with renal dysfunction
139
Q

Reasons to choose CYC over MMF

A
  • IV ensures compliance
  • Intolerance to MMF
  • Caucasian
140
Q

Headache DDX in SLE patient

A

Neuropsychiatric lupus
Aseptic meningitis (from ibuprofen or SLE)
Venous sinus thrombosis
Migraine
Tension headaches
Cluster headaches
Secondary CNS vasculitis
PRES

141
Q

Thrombocytopenia DDX in SLE

A

TTP
Sepsis
ITP
Splenic sequestration
APS
Drug reaction
SLE flare

142
Q

NPSLE PNS manifestations

A

Autonomic disorder
Myasthenia gravis
Neuropathy: cranial, mononeuritis, poly
Plexopathy
GBS

143
Q

Difference between cutaneous DM and SLE skin biopsy

A

Both are vacuolar interface dermatitis

DM inflammation limited to upper dermis
Main difference is depth of infiltrate/mucin deposition

144
Q

Weakness DDX in SLE (Ix, and Tx)

A

Steroid myopathy (CK, EMG, NCS, Pathology –> Taper steroids, PT/OT)
Plaquenil neuromyotoxiciity (CK, biopsy –> stop Plaquenil)
Myalgia/Myositis (BW, Bx –> steroids)

145
Q

Discoid lesion biopsy

A

Hyperkeratosis,
Interface dermatitis w/ lymphohistiocytic infiltrate
Epidermal vacuolation

146
Q

SLE with accelerated atherosclerosis
Give 4-5 factors in SLE which aggravate this

A

GC –> DM, Obesity, and vessel injury
Renal disease –> HTN
Smoking
APS AB
Inflamm (from Type 1 IFN) –> vessel injury

147
Q

How antimalarials help in atherosclerosis

A
  • Decrease production of IL1,6, PG
  • Increase low-density lipoprotein receptors lowering lipid levels.
  • Decrease insulin degradation (help prevent DM)
  • Inhibit plt aggregation/adhesion (help to prevent thrombosis)
148
Q

SLE vs RA hand clinical deformities

A

SLE:
- Nonerosive arthropathy
- Reversible deformities that can become fixed
- Pain/tenderness > swelling
- Predominant tendinitis/tenosynovitis (vs synovitis in RA)

149
Q

SLE vs RA XR deformities

A

SLE:
- No erosions
- No deformities
- Normal joint spaces
- AVN
- Enthesitis more common than in RA

150
Q

SLE vs RA synovial fluid

A

SLE:
-Lymphocyte predominance,
-Fluid only mildly inflammatory,
-Presence of LE cells: neut/macrophages that have engulfed nuclear material

151
Q

4 disease associations with anti-Ro and the frequency of positivity in each disease?

A

SLE (40%), RA (5%), MCTD (Rare), dSSc (10-20%), Primary Sjogren’s (75%)

152
Q

List the disease association with anti-Jo and the frequency?

A

Myositis – Anti-synthetase syndrome ~20-30%

153
Q

Disease associated with anti-histone antibodies and the frequency?

A

Drug-induced lupus –between 90-95%

1. Rheum RC (23 decks)

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