Rheumatology Flashcards
(94 cards)
1
Q
Joint pain:
Monoarticular
Polyarticular (sym)
Polyarticular (asym)
A
Monoarticular
- Septic
- Gout, CPPD
- OA
- Trauma
- Hemarthrosis
Polyarticular (sym)
- RA
- PsA
- PMR
- EA
- AS
Polyarticular (asym)
- Gonococcal
- Lyme
- ARF
- ReA
- Viral
2
Q
Examples of seroNEGATIVE spondyloarthropathies?
A
“PEAR”:
- PsA
- EA
- AS
- ReA
- Undifferentiated
3
Q
Examples of seroPOSITIVE spondyloarthropathies?
A
- RA
- SLE
- Scleroderma
- Sjogren
- Inflam. myopathies (PM/DM)
- Mixed CTD
4
Q
Develop DDx for Joint Pain: articular vs. Non-articular
A
Articular
- Inflam: infectious, post-strep, AI, crystal
- Degenerative: primary (OA), secondary
- Neoplasm
Non-Articular
- Localized Pain: mechnical, neuropathic, vascular
- Generalized Pain: non-inflam, inflam, Psych, endocrine
5
Q
Cardinal features of inflam. arthritis?
A
- Morning stiffness (typically >60m)
- Worse with rest, better with activity
- Night pain (esp. latter half of night)
- Swelling
6
Q
What characterizes the following arthritides: mono, oligo, poly
A
- Mono: 1 joint
- Oligo: 2-4 joints
- Poly: >=5 joints
7
Q
Synovial Fluid Analysis: [1] Non-inflam, [2] inflam/crystals, [3] septic. Comment on fluid appearence, WBC, %PMN, crystals.
A
Non-Inflam
- Fluid: clear
- WBC: <2000
- %PMNs: 50%
- Crystals: no
Inflam
- Fluid: cloudy
- WBC: 10,000-100,000
- %PMNs: >50%
- Crystals: -birefring (gout), +birefring (CPPD)
Septic
- Fluid: cloudy/pus
- WBC: >50,000 [bacterial], 10-30K [fungal/myco]
- %PMNs: 90% [bact.]
- Crystals: +/-
8
Q
Develop DDx approach to mono vs polyarthritis
A
Mono
- Acute: infection, crystal, trauma, new IA
- Chronic: non-inflam (OA), IA, infection (fungal, TB), AVN
Poly
- Acute: infectious (viral, IE), new IA
- Chronic: inflam, crystal, ReA, paraneoplastic
IA: inflam arthritis
Chronic (>6/52), Acute (<6/52)
9
Q
Classic S&S for RA? (joint-wise)
A
- Symmetrical
- Tender, swollen joints: wrists, MCP, PIP
- Morning stiffness
- Joint deformity: swan-neck, boutonniere, hitchiker thumb
- Atlantoaxial subluxation
10
Q
RA DDx?
A
- Other CTD
- HCV/cryo
- IE
- Malignancy (B-cell most common)
- Age
- Normal variation
11
Q
In RA, which test can predict more “erosive” disease if positive prior to arthritis?
A
Anti-CCP
12
Q
RA extra-articular manifestations?
A
Constitutional
- fever, myalgia, fatigue, wt loss
Rheumatoid nodules
- skin: nontender, firm, subQ swelling
- lung: rheum pulm nodules (may have fibrosis and pneumoconiosis [Caplan syndrome])
Cardiac
- pericarditis +/- effusion, myocarditis
- CAD, accelerated atherosclerosis (MI, CVA risk)
Lung
- ILD (NSIP, UIP)
- Pleuritis, pleural effusion (rule out infection)
- Bronchiolotus obliterans
Heme
- ACD, neutropenia, splenomegaly
- Felty’s syndrome = sero+ RA, splenomeg, neutropenia
Neuro
- Carpal tunnel (one of earliest signs)
- C1-2 instability/subluxation = life-threatening
Other
- vasculitis, Raynaud’s
- amyloidosis
- scleritis
- sicca syndrome (dry eyes, mouth)
- Sweet’s syndrome
13
Q
What are symptomatic therapies for RA that are NOT disease modifying?
A
- Steroids (<3/12; use lowest dose for shortest time possible)
- NSAID
- Analgesics
14
Q
What are the long-term, disease modifying, therapies for RA?
A
Step 1: Conventional DMARD
- Low disease activity: hydroxychloroq (PLQ)
- Mod-high: MTX mono (oral>subQ)
- Note: triple (MTX/PLQ/SFZ) no longer recommended
Step 2: biologic or small molecule DMARD
- Use when failed MTX mono
- Usually start with TNFi + con’t MTX
- Dose can be reduced if low-disease or remission >=6/12
15
Q
What are the broad categories of therapies for RA management?
A
Conventional DMARD
- MTX, PLQ, SFZ, Leflunomide
Biologic DMARD
- TNFi: adalimumab (humira), etanercept (enbrel), infliximab (remicade), golimumab (simponi), certolizumab pegol (cimzia
- Tocilizumab (actemra)
- Abatacept (orencia)
- Rituximab
Small molecule/targeted DMARD
- Tofacitinib (xeljanz)
- Baricitinib (olumiant)
- Upadacitinib (rinvoq)
- Apremilast (otezla)
16
Q
Broad S&S of vasculitides?
A
- Constitutional: fever, fatigue, wt loss, anorexia
- Arthralgia, myalgia, arthritis
- Mononeuritis multiplex
- Organ ischemia: mesenteric ischemia, stroke, blindnesss, peripheral neuropathy, GN
- Skin changes: palpable purpura, livedo reticularis, necrotic lesions, infarcts of tips digits
17
Q
Other than vasculitis, what other DDx can cause elevation of p-ANCA?
A
- Crohn’s, UC
- Drugs (PTU, cocaine)
- CTD
- Malignancies
- Infections (HBV, HCV, HIV)
18
Q
eGPA typically presents with which symptoms?
A
- asthma
- allergic rhinitis
- peripheral eosinophilia
- peripheral neuropathy
19
Q
What are the elements part of the Five-Factor Score that should be used to guide therapy in eGPA?
A
- Proteinuria [>1g/d]
- Cr [>138.7]
- GI tract involvment
- Cardiomyopathy
- CNS involvement
1 or more = severe disease
20
Q
Which DMARD should you not start in someone who has CHF?
A
TNFi, may worsen HF - if NYHA III or IV HF
21
Q
Which are main S/E of the following DMARDs? MTX, PLQ, Leflunomide, SSZ
A
MTX
- hepatotox, pancytopenia, PO ulcers, teratogenic
- Rx Folic acid to reduce S/E
PLQ
- retinal tox, photosensitivity
Leflunomide
- GI, hepatoxicity, myelossup, teratogenic
SSZ
- GI tox, HA, rash; CI’d if Sulfa allergy
22
Q
Low-dose MTX toxicity - management: nausea, stomatitis, hepatotox, rash, cytopenias, pneumonitis
A
Nausea:
- increase folic acid to 5mg daily
- trial H2-b/PP
- add leucovorin post-MTX-dosS
Stomatitis:
- increase folic acid
- add leucovorin
- reduce MTX dose if not better
Hepatotox:
- mild - reduce MTX dose
- if >2 ULN - hold MTX then resume a lower dose 1-2 weeks after normalization
Rash:
- dose reduce MTX, d/c if persists
Cytopenias:
- dose reduce or d/c if severe
Pneumonitis:
- d/c MTX, do not restart
23
Q
Low-dose MTX toxicity - management: nausea, stomatitis, hepatotox, rash, cytopenias, pneumonitis
A
- Nausea: increase folic acid to 5mg daily, trial H2-b/PPI; add leucovorin post-MTX-dosS
- Stomatitis: increase folic acid, add leucovorin; reduce MTX dose if not better
- Hepatotox: mild - reduce MTX dose, if >2 ULN - hold MTX then resume a lower dose 1-2 weeks after normalization
- Rash: dose reduce MTX, d/c if persists
- Cytopenias: dose reduce or d/c if severe
- Pneumonitis: d/c MTX, do not restart
24
Q
Keypoints - Biologics: Risks, baseline testing
A
Risks
- infection (new, reactivation)
- drug induced SLE/antibodies
- local skin reactions
- malignancy (esp. non-melanomatous skin Ca)
Testing:
- HBsAg, HBsAb, HBcAb
- HCV (treat concurrently if +ve)
- TST, IGRA, and/or CXR
25
Special situations/controversial use for biologics:
- NYHA III or IV HF
- Active hepatitis
- Prior lymphoproliferative malignancy
- Prior solid organ malignancy
- Prior skin cancer
- Prior serious infection
- Flare: mx of dose/frequency
- TNFi can worsen CHF
- start hepatits tx 1st, consult GI
- use Ritux
- Consult Onco before starting
- csDMARDs preferred
- consider using csDMARDs if serious infection <12mo
- modify frequency rather than dose first
- DO NOT combine biologics
26
Non-live vaccines for patients on RMD? (MTX, Ritux, Pred)
- **MTX:** hold x2/52 after influenza, all other vaccines unchanged
- **Ritux:** time all vaccines when next RTX due, then delay RTX x2/52
- **Prednisone:** give influenza, defer other vaccine if on >20mg until tapered
- **Others**: continue unchanged
27
Seroneg SpA: clinical features
SI joint/axial involvement
Enthesitis, dactylitis, uveitis, conjunctivitis
Peripheral joints:
- **asymmetric, large joint** (AS, PsA, ReA, IBD type 1 [fewer large joints, associated w/ bowel activity])
- **symmetric, small joint** (PsA [DIP], IBD type 2 [many joints, independent bowel])
Skin:
- IBD: erythema nodosum, pyoderma gangrenosum
- ReA: keratoderma blennorrhagicum, circinate balanitis
- Psoriasis: psoriative skin and nail changes
28
Seroneg SpA: imaging features [peripheral, spine, SI joint]
Peripheral XR
- erosions, periosteal new bone formation, ankylosis
Spine XR
- syndesmophytes
SI Joint XR
- sclerosis, narrowing, erosions, ankylosis
- symmetric in AS, asym in PsA
SI Joint MRI
- BM edema
29
Seroneg SpA: Mx of axial disease
Non-Pharm
- PT, exercise
- Quit smoking
Pharm
- 1st-line: NSAID; strongly recommend AGAINST systemic GCs
Step up therapy to Biologics if:
- No response or intolerance to at least 2 different NSAIDs at maximal doses over 1 month, or
- Incomplete response to at least 2 NSAIDs over 2 months
- **1st-line**: TNF-a inhibitors: etanercept (enbrel), infliximab (remicade), adalimumab (humira), certulizumab (cimzia), golimumab (simponi), or biosimilars;
- if primary non-response = progress to IL-17i; if secondary non-response (relapse after initial response) = change to alternae anti-TNF
- **2nd-line**: IL-17i (secukinumab [cosentyx], Ixekizumab [Taltz])
- **3rd-line**: JAKi (tofacitinib [Xeljanz])
30
Seroneg SpA: Mx of peripheral disease
*Non-Pharm*:
- PT, OT
- Weight loss, exercise
- Quit smoking
*Pharm*:
**NSAIDS**
- 1st-line for peripheral arthritis 2/2 AS, ReA
- IBD: use w/ caution, discusss with GI
- PsA: for sx only
**GCs**: avoid if possible, IA inj can be used for mono/oligoarthritis
**DMARD**: MTX, SSZ [Leflu, Cyclo, Apremilast in PsA]
**Biologics/sm**: selected based on disease
31
Seroneg SpA: associate biologic class with typical disease it treats (TNF-a, IL-17, IL-12/23, JAKi, CTLA-4)
- TNF-a: AS, IBD, PsA, ReA
- IL-17: AS, PsA
- IL-12/23: PsA, IBD
- IL-23: PsA
- JAKi: AS, PsA, IBD (UC only)
- CTLA-4: PsA
32
ReA: definition, incubation time, causative agents, joint manifestations, common associated sx (1), treatment
**Definition**: Arthritis s/p gastro or urethritis
**Incubation**: ~4 weeks after infection
**Causative agents**:
- C. trachomatis
- Yersinia
- Salmonella
- Shigella
- Campylobacter
**Joint**: Asym, mono/oligo; lower extremity predominant
**Sx**: 50-75% may have uveitis, conjunctivitis
**Tx**: NSAID, IA steroids
- DMARDs in recurrent/chronic disease (MTX, SSZ, rarely TNFi)
- **NO** role for Abx
33
SLE Classification Criteria? (as per ACR 2019)
ANA titre >=1:80
- If present, apply additive criteria
Additive Criteria (1 clinical + >=10 points)
- See picture below
34
DDx for ANA+
- Rheum: SLE, scleroderma, MCTD, drug-induced lupus, PM/DM, RA
- Thyroid disease, AI hepatitis, PBC, IBD, IPF
- Infection: HCV, Parvo, TB
- FHx of any of the above
- Healthy (healthy titres: 1:40=20%, 1:80=10%, >1:160=5%)
35
SLE Labs?
Which can be used to monitor disease activity?
ANA (sensitive 95%, not specific)
Anti-dsDNA (specific 97-98%, not sensitive)
- Can be used to monitor disease activity along with C3/4 in concordant in dividuals (concordant = when flare, high antids-DNA + low C3/4)
ENA
* Anti-Sm: specific, not sensitive (30-40%)
* Anti-histone: drug0induced lupus, SLE (50-70%)
* Anti-RNP: required for MCTD, SLE (30-40%)
* Anti-Ro (SSA): risk of congenital heart block + neonatal cutaneous lupus
* Anti-La (SSB): Sjogren's
36
Rashes: Malar vs. Rosacea
Malar
- Last few days-weeks
- Spares nasolabial folds
- Precipiated by sun exposure
- Look for other systemic manifestations!!!
Rosacea
- Frequent flushing (last few hours)
- Telangectasia, papules, pustules
- Aggravated by sun, spicy foods/drinks, EtOH
- Can cross nasolabial fold
37
What are the 5 types of Lupus Nephritis + general Mx?
Class I: minimal mesangial
- Supportive +/- immunosuppression if proteinuria >3g/d
Class II: mesangial proliferative
- **Aggressive immunosuppression**
Class III: focal (<50% golmeruli)
- **Aggressive immunosuppression**
Class IV: diffuse (>=50% glomeruli)
- Anti-proteinuric/HTN agents +/- immunosuppression if refractory
Class V: membranous
- Supportive +/- treat extrarenal manifestations
38
Overview of Lupus Nephritis Management? (Class III/IV)
**Induction:**
- Pulse steroids [IV GC 250-500mg/d x3d then Pred 0.6-1mg/kg/d] + 1 other (Cyclo, MMF)
- Cyclophosphamide: low-dose preferred (500mg q2wk x6)
**Risks**: infertility, infection, malig esp. GU (+++ hydration), cytopenias
- MMF: 2-3g/d x6 months *(preferred if considering future fertility)*
**Risks**: GI S/E, cytopenias, unsafe in pregnancy
- **HCQ** for all; *ACEi for proteinuria*
**Maintenance:**
- HCQ + MMF 1-2g/d +/- low dose Pred (target <7.5mg/d by 3 months)
- *Alternative to MMF*: AZA (if preg plans or intolerant), Tacro
39
Class V (membranous) Lupus Nephritis typically does not progress to renal failure UNLESS? % risk of progression? Mx?
- Nephrotic range proteinuria
- 10-30%
- Same as Class III/IV
40
Management of non-renal SLE: All, mild, mod, severe manifestations?
**All**
- sun protection
- vaccines, smoking cessation, exercise
- BMI, BP, lipids, glucose
- anticoagulation/anti-platelet if aPL+
**Mild** (constitutional sx, mild arthritis, rash, plt 50-100)
- HCQ +/- GC (PO/IV) + topicals (GC, retinoids) for skin
- *refractory*: add MTX/AZA
**Moderate** (RA-like arthritis, cutaneous vasculitis, plt 20-50, serositis)
- HCQ +/- GC (PO/IV) +/- MTX/AZA
- *refractory*: belimumab, calcineurin inhibitor, MMF
**Severe** (organ threatening disease [nephritis, cerebritis, myelitis, penumonitis, mesenteric vasculitis], plt <20, TTP-like disease, AIHA)
- HCQ +/- GC (PO/IV) +/- MMF or Cyclo
- *refractory*: Cyclo, Ritux
41
Anti-phospholipid criteria?
**Criteria:** clinic + laboratory criteria
Clinical:
- *Vascular thrombosis*: >=1 episode of arterial, venous, small vessel
**OR**
- *Pregnancy*: >=1 unexplained death of normal detus >10 GA **or** >=1 premature delivery of normal fetus < 34 GA bc pre/eclampsia **or** placental insufficiency **or** >=3 unexplained consecutive miscarriages <10wk
Lab: 2 positive results >12wk apart and no more than 5yr prior to S&S
- *Lupus Anticoagulant* **(most powerful predictor of thrombosis)**: DOACS can cause false positive LAC; warfarin does not interfere
- *Anticardiolipin-Ab* (IgM, IgG): no affected by anticoagulation
- *Anti-B2 Glycoprotein I*: not affected by anticoagulation
42
Lupus & Pregnancy - Mx considerations for the following: SLE, Ro/La+, aPL+
**SLE**
- Continue HCQ during pregnancy
- Start ASA 81 daily prior to 16 GA [reduce risk pre-eclampsia]
**Ro/La+**
- *No hx neonatal lupus*: HCQ + serial fetal echo from 16-26 GA
- *Hx neonatal lupus*: HCQ + serial echo weekly from 16-26 GA
**aPL+**
- *No APS*: ASA alone
- *OB APS*: ASA + propjhylactic heparing until 6-12wk P-P
- *Thrombotic APS*: ASA + therapeutic heparin during preg + P-P
43
SLE Trivia:
- Drug-induced lupus: examples, serology
- Shrinking Lung Syndrome: definition, imaging findings
- Libman Sacks Endocarditis: associated with which disease, risks, tx
**DIL**
- Hydralaine, procainamide, TNFi, isoniazid
- ANA+, dsDNA-, anti-histone-Ab+
**SLS**
- rare complication r/t diaphragmatic muscle weakness
- lungs clear on imaging, but volume decreased (CXR, MIP/MEPs)
**LSE (non-infectious)**
- associated with APLAS
- thrombus on valve consists of accumulations of immune complexes, mononuclear cells, hematoxylin bodies, fibrin/plt thrombi
- can result in embolic phenomena
- steroids, anticoagulation
44
DDx for bilateral parotid gland enlargment?
* Sjogren's
* Infectious: mumps, TB, bacterial
* Sarcoidosis, IgG4 syndrome
* Lymphoma
* Alcoholism, anorexia/bulimia
45
Sjogren's: S&S, Serology, Epi, Dx
S&S
- Xerostomia, keratoconjunctivitis
- *Non-glandular*: arthritis, **vasculitis**, demyelinating neuropathy, RTA
Serology: ANA, **Anti-Ro/La**, RF
Epidemiology:
- **>40x increased risk of B-cell lymphoma**
- Can occure with SLE, RA
Diagnosis:
- Ophtho: Schirmer's test [<5mm in 5 min]
- Unstimulated salivary flow
- ENT: minor salivary gland biopsy = focal lymphocytis sialadenitis
46
Systemic Sclerosis (scleroderma): S&S
- Diffuse
- Limited cutaneous/CREST Syndrome
- Serology: tests, which are useful for disease monitoring
**Diffuse**
- Sclerodactyly proximal to elbows/knees
- Internal organ involvement ***(risk of ILD, renal crisis)***
**Limited/CREST**
- Calcinosis
- Raynaud's
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasias
- *pHTN in up to 5%*
**Serology**
- anti-centromere: **CREST (60%)**, diffuse (15%)
- anti-Scl-70/topo I: ~40% of scleroderma patients; **mostly diffuse**
- neither useful for disease monitoring
47
Complications of Systemic Sclerosis: name 3
- Renal crisis
- pHTN
- Gastric antral vascular etasis (results in GIB that can be life-threatening)
48
Complications of Systemic Sclerosis:
- **Renal Crisis**: more common in which form, risk fx, clinical signs, tx
- **pHTN**: more common in, monitoring
- Gastric antral vascular ectasis
**Renal Crisis**
- 10-20% **diffuse** systemic sclerosis
- Increased risk: pred, RNAP3-Ab, early disease
- S&S: renal failure, HTN, mild proteinuria - but can be normotensive
- Tx: Captopril (ACEi)
**pHTN**
- 5-19% of all systemic sclerosis; more common in **limited/CREST**
- Monitor: BNP, echo, PFTs *annually*
49
Scleroderma Renal Crisis vs. TTP: S&S, Hx, Labs, Tx
**S&S**
- SRC: SOB, aLOC, HTN (not always), **S&S scleroderma**
- TTP: fever, HTN, purpuric rash, bleeding, neuro S&S
**Typical Hx**
- SRC: MAHA, AKI, proteinuria, hematuria
- TTP: MAHA, **thrombocytopenia**, AKI, proteinuria, hematuria
**Labs**
- SRC: Anti-Scl-70/TopoI, Anti-centromere
- TTP: ADAMTS13 (low)
**Treatment**
- SRC: captopril
- TTP: PLEX, steroids, Ritux
50
Secondary causes of Raynaud's Phenomenon?
- Drugs: bb, ergotamine, bleomycin
- Infection: HBV, HCV, Parvo B19
- Smoking
- Occup trauma: from handling vibrating tools, typing
- Hyperviscocity: polycythemia, paraproteinemias (plasmacytoma, Waldenstrom), cryo, cold agglutinin disease
- Vasculitides (eg., Buerger's)
- CTD: scleroderma, CREST, SLE, MCTD
- HypoT4, Carcinoid, PCC
- Heme malignancy
51
Raynaud's Phenomenon: Epi & S&S - primary vs. secondary
Primary
- F>M, onset ~20yo, often FHx
- Sym., typically not progressive
- Predictable onset (cold)
- **ANA negative**
Secondary
- Males, >40s
- Asym., can be progressive
- **Abnormal nail folds**
- Pits and ulceration of digits
52
Raynaud's Phenomenon: Labs, Treatment
**Labs**
- *Only if suspicious of secondary*
- CBC, Cr, U/A, LFT, CK, ESR, CRP
- ANA, ENA, RF, C3/4, Cryo
- TSH, SPEP/UPEP
- HBV, HCV
- PTT, APLA
**Treatment**
- Conservative
- *1st line*: **CCB**
- Can consider topical nitrates, PDE5i
53
DDx Myopathy
Drugs:
- statins, anti-psych, colchicine, anti-retrovirals, Li, SSRIs
Idiopathic myopathy
- PM/DM/IBM/NAM
Infectious
- Viral: HIV, EBV, CMV, inluenza
- Pyomyositis
Hypothyroid myopathy
Electrolytes disorders [hypoK/Phos]
Genetic myopathy
54
DM/PM Clinical Features: [1] muscle weakness [2] cardiac [3] pulmonary [4] skin
Muscle Weakness
- Insidious over weeks/months
- Symmetric; proximal > distal
- Can involve: heart, diaphragm, oropharynx, esophagus
Cardiac
- myocarditis, arrhythmia, CHF
Pulmonary
- ILD (NSIP, UIP), DLCO or CT abn, pHTN
Skin
- Gottron's
- Shawl sign
- Heliotrope rash
- Generalized erythroderma
- Periungal erythema
- Mechanic's hand
- Scalp psoriasiform changes
- Calcinosis cutis
55
Cutaneous manifestations of DM/PM?
- Gottron's papules
- Heliotrope rash
- Shawl sign
- Generalized erythroderma
- Periungal erytherma
- Mechanic's hand
- Scalp psoriasisform changes
- Calcinosis cutis
- Nailfold capillary dilatations
56
DM/PM: Investigations?
- Labs: **CK**, AST, LDH, ESR, CRP, **myositis panel**, ANA, ENA; trop, ECG, +/- Echo to r/o cardiac
- **MRI muscle**
- **Muscle biopsy (gold standard)**
- Muscle EMG: irritability, low amp, repetitive dc
- SLP assessment: r/o oropharyngeal/esoph involvement
- Spirometry w/ MIP/MEPs: r/o diaphragmatic involevment
- Age appropriate cancer screening: consider CT c/a/p
57
What is typically present at the time of Dermatomyositis diagnosis or within 1 year of diagnosis?
Malignancy
58
Myositis panel associated with which antibodies/diseases?
Anti-Jo1-Ab
- **Anti-synthetase syndrome**
- Acute onset, constitutional sx
- Rapidly progressive ILD
- Raynaud's phenomenon, mechanic's hand, skin ulceration, arthritis
Anti-Mi2-Ab
- Associated with classic form of **DM**
- Highly responsive to treatment; favourable prognosis
Anti-NXP2 & Anti-TIF1-y-Ab
- Highly associated with **malignancy**
59
DM/PM: Management [1st line, refractory]
**1st line**
- **High dose steroids** (at least 1mg/kg): usually PO; IV pulse if severe
- **Steroid sparing agent**: MTX or AZA [HCQ helpful for *skin S&S only*; MMF/Cyclo *if ILD*]
**Refractory or Severe**
- IVIG
- Rituximab
Continue routine, age-appropriate cancer screening as *risk of malignancy high for at least 5 years after DM diagnosis*
60
IBM vs. DM/PM: weakness, CK
**IBM**
- Distal > proximal
- CK tends to be lower
- Poor treatment response
**DM/PM**
- Proximal > distal
- CK tends to be higher (usually)
61
What is Necrotizing Autoimmune Myositis? (NAM; definition, S&S, investigations)
Defintion
- Inflam myopathy affecting prox. muscles
- CK +++ high
- Persistent after d/c Statin
S&S
- Weakness: **severe**
- Progression: weeks/months
- Skin/systemic features: **rare**
Investigations
- CK (+++ high), ESR/CRP (N/mildly high), TSH, HBV, HCV, HIV
- Anti-HMG CoA reducatase Antibody
- Rule out paraneoplastic syndrome
62
Septic Arthritis - Most common infection in:
- native/prosthetic joints
- OM and septic arthritis in Sickle CD
- Can crystal arthritis co-exicst wit infection?
- S. Aureus
- Salmonella
- Yes - do not be fooled!
63
Gonococcal Arthritis: what are the 2 common syndromes? Tx? Which syndrome requires longer treatment?
Syndromes:
1. **Triad**: tenosynovitis, vesiculopustular skin lesions, migratory polyarthralgias w/o purulent arthritis
2. **Purulent arthritis** w/o skin lesions
Treatment
- Ceftriaxone
- Treat CT empirically or if concurrent
- Syndrome #2 requires longer course Abx
64
Gout vs CPPD: crystals
- Gout: -ve birefringence
- CPPD: +ve birefringence
65
Gout Tx: acute vs chronic
**Acute:**
- *NSAIDs*
- *Colchicine*: 1.2mg load, then 0.6mg an hour later, then 0.6mg BID until sx resolution
- *GC* (IA/PO): IA if monoarthritis, PO if poly (or C/I to above meds)
- *Il-1 blocker (anakinra)*: consider only in pts with frequent flares and CI to colchicine, NSAID, GC
**Chronic:**
*Non-Pharm*:
- Exercise, wt loss
- Avoid EtOH, sugar-sweetened drinks, excessive meat/seafood
*Pharm*: urate-lowering meds
Definite Indications:
- >=2 attacks/yr
- Tophaceous gout
- Gouty arthropathy (eg, erosions)
Conditional indications: 1 gout attack +
- CKD stage III+
- Uric acid >535
- Urolithiasis
Tx Options:
- 1st line: Allopurinol (start 100mg/d)
- Febuxostat 80mg/d (if cannot tolerate Allo)
- Oveerlap with anti-inflam proph w/ either colchicine, NSAID, or low-dose GC for 3-6 months
66
Gout - Allopurinol:
- If flare, but on chronic Allo - what to do?
- Can initiate Allo during acute flare?
- Continue Allopurinol
- Yes, can overlap with NSAIDor Colchicine or low-dose GC for 3-6 months
67
Vasculitides - Name examples of the following: small, medium, large, variable
**Small**
- GPA [Wegener's]
- eGPA [Churg-Strauss]
- MPA [microscopic polyangiitis]
- HSP [Henoch-Schonlein Purpura]
- Anti-GBM, Goodpasture
- Cryoglobulinemia Vasculitis
- Cutaneous Leukocytoclastic
**Medium**
- PAN
- Thromboangiitis obliterans [Buerger's]
**Large**
- GCA
- Takayasu
**Variable**
- Behcet's
- Cogan's
68
GCA: name cranial + extracranial S&S
**Cranial**
- HA
- Jaw claudication
- Diplopia
- Scalp tenderness
- Amarausis Fugax
- Stroke (mostly posterior)
**Extra-cranial**
- Limb claudication (upper/lower)
- Constitutional symptoms
69
Name a few exam findingsof GCA
- Beaded, prominent or tender temporal arteries
- Absent temporal artery pulses
- Fundoscopy: acute ischemic optic neuritis
70
ACR/EUCLAR 2022 diagnostic criteria for GCA?
50yo, **AND**
Score >=6
71
GCA: Management
- Visual symptoms/loss or critical cranial ischemia
- No visual sx/loss or critical cranial ischemia
- Extracranial GCA
- ASA?
1. IV pulse steroids 1g x3d then Pred 1mg/kg/day + Tocilizumab (TCZ)
2. Pred 1mg/kg/day + TCZ
3. Pred 1mg/kg/day + TCZ or MTX
4. ASA: only if critical/flow-limiting lesion of carotid/vertebral arteries
Overall: high-dose steroids x1 month, then taper
72
PMR diagnosis criteria?
***Required**
- ***Age >50yo**
- ***High ESR or CRP**
- ***Bilateral shoulder ache +/- hip pain stiffness**
- Negative RF, CCP, CK [if high, think myositis]
- U/S: shoulder/hip bursitis, tenosynovitis, synovitis
- Morning stiffness >45m
73
PMR management?
- Prednisone 12.5-20mg/d x2-4 weeks, then taper to 10mg/d within 1-2 months if response
- *If relapse*: increase to pre-relapse dose, then decrease gradually and/or add DMARD (eg., MTX)
74
PAN:
- Definition
- S&S
**Definition**
- Medium vessel vasculitis leading to tissue ischemia
- Can be local or systemic
**S&S**
- Constitutional, wt loss >4kg
- Skin: nodules, ulcers, livedo reticularis
- Testicular pain
- Abdo pain (post-prandial)
- Myalgias (exclu. shoulders/hip girdle)
- Inflam. arthritis
- Mono/polyneuropathy or ***mononeuritis multiplex***
- Arteriographic abn: aneurysms or stenotic lesions in mesenteric/hepatic/renal arteries + branches
- +/- HBV infection
75
PAN:
- Diagnosis
- Treatment
**Diagnosis**
- Biopsy: mixed inflam cells in vessel wall + fibrinoid necrosis; **no** granulomas or giant cells
- Abdo CTA/MRA: determine extent of disease
Treatment
**Treatment**
1. If HBV-related: GC, antivirals +/- PLEX
2. If idiopathic:
- *Non-severe* [mild systemic sx, uncomplicated skin, arthritis]: GC + MTX or AZA
- *Severe* [renal, neuro, limb/cardiac/mesenteric ischemia]: pulse steroids x3-5d then Pred 1mg/kg/day + Cyclosphosphamide [PO/IV] x3-6 motnhs
76
Name a few ANCA-associated small vessel vasculitides + if p- vs c- ANCA
GPA (Wegener's)
- c-ANCA (PR3) [80%]
EGPA (Churg-Strauss)
- p-ANCA (MPO) [40%]
- Px as: ashtma, allergic rhinitis, peripheral eosinophilia + neuropathy
MPA
- c-ANCA [30%], p-ANCA [65%]
77
Causes of p-ANCA (MPO) elevation other than vasculitis [eg., eGPA, MPA]
- IBD
- Drug [PTU, cocaine]
- CTD
- Malignancies
- Infections [HBV, HCV, HIV]
78
Name ANCA-associated vasculitides symptoms
- Constitutional symptoms, wt loss
- ENT: nasal crusting, sinusitis (more common in GPA)
- Tracheal + pulm involvement: senosis, hemoptysis
- Renal: RPGN (pauci-immune), hematuria, proteinuria
- Pulm-renal syndrome
- Cutaneous vasculitis
- Mononeuritis multiplex
79
Management of GPA/MPA: [1] **Induction** [2] Maintenance
Induction: Severe Disease
- Pulse steroids x3-5d then reduce dose taper **AND** Rituximab
- Cyclophosphamide: if CI'd/failed Ritux
- PLEX: recommend *against* in pulm hemorrhage and in RPGN; recommend *for* concurrent anti-GBM
Induction: Non-Severe Disease
- GC + MTX
80
Management of GPA/MPA: [1] Induction **[2] Maintenance**
Severe:
- GC: reduced dose taper, **PLUS**
- Ritux q4-6mo > MTX or AZA > MMF or Leflunomide
- PJP prophylaxis in all receiving RTX of CYC
Non-Severe:
- Remission induced with MTZ, AZA, or MMF: continue on same meds
- Remission induced with RTX, CYC: consider RTX, MTZ, AZA, LEF
GC should be guided by S&S. Non-GC agents typically used for >=18mo
81
GPA/MPA [1] Relapse [2] Refractory: Management
Relapse
- If not on RTX for maintenance: RTX
- On RTX for maintenance: CYC
Refractory
- Switch agents if refractory to RTX or CYC
- Add IVIG to remission induction
82
eGPA: Management **[1] induction** [2] maintenance
Severe Disease:
- GC IV pulse or high-dose oral [1mg/kg] **PLUS**
- CYC or RTX
Non-Severe (asthma, non-severe vasculitis):
- GC **PLUS**
- Mepolizumab > MTX or AZA > CYC or RTX
83
eGPA: Management [1] induction **[2] maintenance**
Severe
- GC + MTX or AZA or MMF
- Optimal duration GC tx unknown, may require ongoing low-dose GC to control asthma/allergy symptoms
84
What are the 5-Factor Score used to guide therapy in eGPA?
- Proteinuria >1g/d
- Creat >138.7
- GI tract involvememt
- Cardiomyopathy
- CNS involvement
**Score >=1: severe disease**
85
eGPA: Management - Relapse
- *If severe*, previously induced CYC: RTX
- *If severe*, previously induced RTX: CYC
- *If non-severe* while on MTZ/AZA/MMF/GC mono: Add Mepolizumab
86
Cryoglobulinemic Vasculitis:
- Definition
- Classification
Definition:
- Vasculitis caused by deposition of temperature-dependent IgG/IgM Ig/immune complexes (eg., cryoglobulins)
Classification:
- **Type I**: monoclonal - seen in clonal heme disease (MGUS, MM, CLL, WM); *S&S r/t vascular occlusion [small vessel]*
- **Type II**: mixed - can be 2/2 chronic infection [HBV HCV, HIV, IE], CTD, lymphoproliferative disorders; *S&S r/t small [rarely medium] vasculitis*
87
Cryoglobulinemic Vasculitis: Clinical presentation
**Asymptomatic**: +ve cryglobulins w/o EOD
**Symptomatic**: +ve cryo + EOD
- *Mild/mod*: non-ulcerating skin lesions [purpura, acrocyanosis, livedo reticularis], non-debilatating peripheral neuropathy, arthralgias/arthritis
- *Severe*: cutaneous ulcers, progressive/debilatating meuropathy, GN with renal failure or nephrotic syndrome
- *Life-threatening*: RPGN, CNS involvement, intestinal ischemia, alveolar hemorrhage
88
Cryoglobulinemic Vasculitis: Management - HCV associated vs. Non-Infectious
**HCV**
*Mild/mod:*
- Induction: antivirals +/- GC
- Maintenance: antivirals
*Severe:*
- Inducation: RTX + GC
- Maintenance: antivirals
*Life-threatening:*
- Inducation: PLEX + GC pulse + RTX or CYC
- Maintenance: antivirals
**Non-Infectious**
*Mild/mod:*
- Treat underlying disease [if identified]
- If no disease identified, low-dose GC or colchicine
*Severe:*
- Inducation: RTX or CYC + GC
- Maintenance: treat underlying disease + lowest effective immunosuppression
*Life-threatening:*
- Inducation: PLEX + GC pulse + RTX or CYC [if RTX failed or unavailable]
- Maintenance: treat underlying disease + lowest effective immunosuppression
89
Non-pharm Management of OA:
- General
- Hip/Knee
- Hand
**General**
- Exercise
- Self efficacy/mx program
- consider CBT, thermal interventions, acupuncture
**Hip/Knee**
- Wt loss
- Tai Chi
- Cane
- TF knee brace [knee only]
- Balance training, Yoga, PF knee brace, radiofrequency ablation
- *TENS* **strongly recommended AGAINST**
**Hand**
- 1st CMC orthosis
- Other hand orthoses
- Paraffin
90
Pharmacologic Management of OA [hand, hips, knee]
**Use:**
- PO NSAIDs if no CI's
- Topical NSAIDs
- IA GC injections
- Consider Tylenol, duloxetine, chondroitin *[hand OA]*, topical capsicin *[knee/hip]*, tramadol
**DON'T use:**
- Opioids
- Glucosamine
- DMARD/Biologic
- Bisphosphonate
- Platelet rich plasma, stem cell injections, IA hyaluronic acid
- Chondroitin for knee/hip
91
If prescribing Tramadol - why shoud be cautious about it?
1. CYP2D6 metabolized leading to high variability between patients
2. Caution if:
- Other SSRI [serotonin syndrome]
- Hypoglycemia history
- Seizure history
92
Indications + CI [absolute, relative] for IA GC injections?
**Indications:**
- Knee OA, especially if with effusion
- Crystal arthritis [CPPD, gout]
- Inflam. arthritis [eg., RA]
**Contraindications:**
*Absolute:*
- Possible septic arthritis
- Prosthetic joing
- Overlying cellulitis or suspected bacteremia
- Allergy to GC
*Relative:*
- Brittle diabetes
- Clotting/bleeding diasthesis or anti-thrombotic meds
- Failure to respond to previous IA GC injections
93
Fibromyalgia: [1] diagnostic criteria [2] screening labs [3] Rx
**Diagnosis:**
- Widespread pain index + symptom severity scale
- Symptoms present + similar level >3 months
- No other disorder to explain symptoms
- Patients often c/o: fatigue, cognitive dysfunc., non-restorative sleep
- Tender points *no longer* in diagnostic criteria!
**Screening Labs:**
- CBC< ESR, CRP, CK, TSH = all **MUST** be normal
**Rx:**
- Non-pharm: exercise, CBT, sleep, Tai Chi
- Pharm: SNRI, TCA, gabapentin
94
CV Risk Reduction - Patients with Rheumatic diseases have increased CV risk compared to general population. What are special considerations for the following:
- RA
- Gout
- SLE
- APS
**RA**
- Total cholesterol & HDL-c should be used in risk factor prediction [TG, LDL-c tend to be lower]
**Gout**
- Serum uric acid < 0.36 can potentially lower CV event risk/mortality
- No preference between allopurinol/febuxostat
- *Avoid*: diuretics, favour CCB or Losartan [uricosuric]
**SLE**
- BP < 130/80 considered in ALL patients with SLE
- ACEi/ARB Rx'd to those w/ lupus nephritis, urine PCR >500mg/g, or HTN
- Tx with HCQ may reduce CV event risk
**APS**
Low-dose ASA recommended for:
- Asymptomatic aPL carriers with high-risk profile
- SLE patient with high-risk aPL profile but no APS
- Considered for SLE patient with low-risk aPL profile
