Clinical Medicine III: Block I, MSK Objectives 7: Rheumatology Jimmy Bui

RHEUMATOLOGY

READING: Cecil Chapters 80 [pages 495-496], 264, 265, 266, 267, 273, 274, 275, 276, 277, 278, 279 & 282; EMC pages 45-51, 858-860, 354-357 & 419; | Porth Chapter 43, pages 1119-1131

Fast Facts Drugs Rheumatology: field that deals with dz involving joints, autoimmunity or both Inflammatory arthrides: RA, spondyloarthritis, infn, crystal-induced

o Aggravation c/ rest, alleviated c/ use, warmth, redness of jts + constitutional sx (e.g. wt loss, fever, fatigue

Non-inflammatory arthrides: OA, trauma, hemarthrosiso Aggravation c/ motion, alleviated c/ rest

Kills inflammatory cells – methotrexate, cyclophosphamide, azathioprine, leflunomide, mycophenolic acid

Prevent cytokine secretion – cyclosporine (inhibits t-lympocytes and their interleukins), corticosteroids

Inhibit cytokine synthesis – NSAIDs, COX-2 inhibitors Block cytokines – anti-TNF Ab

1. Cite indications for the following laboratory studies: ESR, CRP, ANA, c-ANCA, p-ANCA, anti-DNA antibodies, serum complement, Cryoglobulins.

Laboratory Studies IndicationsESR/CRP ANA c-ANCA p-ANCA Anti-DNA Ab Serum Complement Cryoglobulins

↑ d/t inflamm Antinuclear Ab (+) in 40-60% RA pts

Cytoplasmic Antineutrophil Cytoplasmic Ab

Wegener’s granulomatosis

Perinuclear Antineutrophil cytoplasmic Ab

↑ in Polyarteritis nodosa

Serologic marker for SLE High levels reflect acute inflamm

Ig’s that precipate in cold & promote systemic inflamm dz via tissue deposition

2. Differentiate between primary and secondary Raynaud’s phenomenon. Capillary spasms resulting in loss of blood flow that are exacerbated in cold exposure (any part of the body) Effected digits turn white → blue → red

i. Tx: SL or use CCB (but may ↓ BP) 1° : no underlying cause

i. PE is nL between attacks 2° : associated c/ or caused by some other systemic illness of dz process

i. pits/ulcerations on fingertips may be present in pts c/ scleroderma CREST syndrome (Calcinosis, Raynaud’s syndrome, Esophageal dysmotility, Sclerodactyly, Telangiectasias)ii. abnL Allen test indicates fixd arterial occlusion

3. Compare and contrast the epidemiology and pathophysiology of the following categories: autoimmune diseases, vasculitic syndromes, and seronegative spondyloarthropathies. Refer to Table Below4. Describe the characteristic clinical presentation, diagnosis, management, prognosis/characteristic disease course and major complications of the following diseases: Refer to Table Below

∴ therefore, ∵ because, c/ with, s/ without, MCC: Most Common Cause 1

Clinical Medicine III: Block I, MSK Objectives 7: Rheumatology Jimmy Bui

Autoimmune Diseases and Connective Tissue DisordersDisorder Features SSX DX TX/PX

Systemic Lupus Erythematosus

(SLE)

Epi: MC in 20-30s 3x F M≫ AA White≫

1) Malar (butterfly) rash (s/ scarring) triggered by sun exposure d/t photosensitivity

2) Discoid lupus: rash occurs in diff parts of bodies that can result in scarring

3) Subacute cutaneousa. Annularb. Serpinginous

Alopecia (reversible) Arthritis esp small jts of hand Renal : GN, membranous nephritis,, mesangial nephritis Pulm : pleuritic, pneumonitis, interstitial lung dz (pulm fibrosis) Cardiac : pericarditis, myocarditis, coronary vasculitis, CAD, Libman-

Sacks endocarditis (growths on heart valves) Heme : leukopenia, lymphopenia, TP, (+) Coombs hemolytic anemia,

hypercoagulable CNS : peripheral neuropathy, Sz, CVA, organic brain syndrome

(dementia-like), HA, depression, psychosis Ocular : conjunctivitis, episcleritis, Sjögren’s syndrome, CRAO, cytoid

bodies (rare s/ affecting vision)

SLE is dx’d by clinical criteria (Need 4/11)1) Malar rash2) Discoid Rash3) Photosensitivity4) Oral ulcers5) Arthritis6) Serositis7) Renal d/o8) Neuro d/o9) Heme d/o (only 1 pt despite

having several)10) Immunological d/o11) Antinuclear d/o

ANA Test is TERRIBLE for screening SLE

Most valuable if someone has 3 other features of SLE

Skin : hydroxychloroquine (old malaria drug), topical steroids

Jt : NSAIDs, hydroxychloroquine Renal , CNS: corticosteroids,

cyclophosphamide, azathioprine, mycophenolate mofetil, rituximab

Heme : : corticosteroid Serositis : NSAIDs, corticosteroid Steroid sparing : methotrexate,

azathioprine, mycophenolate mofetil, belimumab (newer monoclonal Ab)

Systemic Sclerosis /

Scleroderma

Def Deposition of collagen into skin Often starts off with Raynaud’s

phenomenonEpi 4x F M≫ 40-60yr No hereditary component

Edema of hands Shiny skin Skin pulled tight → fingers pulled into

claw-form Telangioectasia Digital ulcerations @ tips (feels like worst

paper cuts) Interstitial lung dz (aveolar scarring) →

often cause of death Basically CREST syndrome

(+) ANA 90% cases Skin: corticosteroids, peniciliamine, topical NTG

Jt, NSAIDs, corticosteroid Esophagus: PPI Raynaud’s: CCB Renal: ACEi Pulm: corticosteroid,

cyclophosphamide, bosetan Bact overgrowth: broad-spectrum Abx

Sjögren Syndrome

Def: Autoimmune destruction of salivary &

lacrimal glands (exocrine)Epi: F M, middle age≫

Dry eyes (keratoconjunctivitis sicca/xerophthalmia)

Dry mouth (xerostoma) ± salivary gland enlargement May be associated c/ rheumatoid arthritis

or other connective tissue dz Enlarged parotid glands Dry nasal passages, bronchi, cough, skin,

vag

Schirmer’s test: filter paper in eyelid to see tear production

↓ salivary gland flow salivary gland bx → lymphocytes in

glands RF in 70% cases ANA in 60% cases

Dry eyes: Artificial tears, punctal occlusion, cyclosporine

Dry mouth: sugarless lozenges, pilocarpine, cevimeline

Polymyositis Proximal mm weakness (difficulty

getting out of chair & brushing hair)Often painless

Epi 3x F M≫

Proximal mm weakness (difficulty getting out of chair & brushing hair)

Often painless

↑ CPK Myopathic ∆s on EMG Muscle bx → inflamm ∆s

HD Corticosteroid Methotrexate Azathioprine IVIG

Dermatomyositis

Derm manifestations: Heliotrope rash (around eyes) “mechanic’s hands” (hyperkeratosis of

hands) Gottron’s papules over jts

Polymyalgia Rheumatica

(PMR)

Epi 2x F M, ≥ 50yo, White≫ Associated c/ Temporal arteritis

MM pn x ≥ 1mo of shoulders, pelvic girdle Sev morning stiffness & gelling No mm atrophy or true weakness Constitution sx (fever, fatigue, wt loss,

depression)

↑ ESR >40mm/hr or ≥ 100mm/Hr bx for temporal arteritis

LD Corticosteroid x 2yr c/ tapering HD Corticosteroid if (+) temporal

arteritis

∴ therefore, ∵ because, c/ with, s/ without, MCC: Most Common Cause 2

Clinical Medicine III: Block I, MSK Objectives 7: Rheumatology Jimmy Bui

Vascular SyndromesDisorder Features SSX DX TX/PX

Giant Cell Arteritis(Temporal Arteritis)

Inflammation of branches of temporal artery

Local Temporal HA Blindness Scalp necrosis Jaw claudication Neuropathies (CNS,

PNS)

Systemic PMR Fever Anorexia Malaise Wt loss

↑ ESR, abnL LFT, anemia ACR Criteria (need 3/5) Age >50 y/o @ onset New HA Temporal artery: ↓ pulse, TTP Bx → granulomatous inflamm c/

multinucleated giant cells

Corticosteroid Methotrexate Azathioprine

Takayasu’s Arteritis

Def Arteritis of branches of aorta RareEpi F M, 10-40 y/o≫ MC in Japanese

↓ pulses in extremities Cool extremities Claudication

ACR Criteria (need 3/6)1) Age < 40 y/o @ onset2) Claudication3) ↓ brachial artery pulse4) BP diff > 10mmHg b/t arms5) Bruit in subclavian aa or aorta6) Occlusion/narrowing of aortic branches

Corticosteroid Methotrexate Surgical revascularization

Polyarteritis Nodosa

Systemic necrotizing arteritis of medium aaEpi 3x M≫F, 40-60 y/o Associated c/ Hep B (30% cases)

Livedo reticularis Inability to extend hand Mesenteric aa → necrosis of gut, kidneys

Definitive dx: Bx angiogram ↑ESR, CRP, proteinuria (+) Hep B Ag

HD Corticosteroid Cyclophosphamide ACEi

Wegener’s Granulomatosis

(Granulomatosis c/ polyarthritis)

Vasculitis of small & medium vessels

Sinus, pulm, renal involvement

ACR Criteria (need 2/4) Nasal/oral inflame (bloody nasal d/c, oral

ulcer) AbnL CXR (nodules, infiltrates, cavities) Ur sediments (>5 RBC/casts) Bx → granulomatous inflamm

Corticosteroid Cyclophosphamide Limited dz →

methotrexate, azathioprine, TMP/SMZ

Behçet’s Syndrome

Recurrent oral & genital aphthous ulcers

Uveitis→ blindness

Recurrent oral & genital aphthous ulcers Uveitis→ blindness Skin ulcers

Recurrent oral ulcers 2 of following s/ systemic dz:

o recurrent genital ulcero uveitiso skin lesiono (+) pathergy test (papule ≥2mm dev

24-48hr post-oblique insertion of 20-25 gauge needle into skin)

Colchicine Corticosteroid NSAIDs (for jts) Methotraxate Azthioprine Cyclophosphamide

Vasculitis ClassificationArtery Size Clinical Syndrome

Large artery Takayasu’s arteritisLarge & medium artery Giant cell arteritisMedium sized artery Polyarteritis nodosum, Wegener’s granuloma-tosus, CT diseaseSmall vessels Hypersensitivity vasculitis, Henoch-Schönlein purpura, CT disease

∴ therefore, ∵ because, c/ with, s/ without, MCC: Most Common Cause 3

Clinical Medicine III: Block I, MSK Objectives 7: Rheumatology Jimmy Bui

Seronegative SpondyloarthropathiesDisorder Features SSX DX TX/PX

Ankylosing Spondylitis

Onset <40yo Insidious onset Duration >3mo Morning stiffness Improvement c/ exercise

Bilat sacroiliitis Spondylitis Peripheral oligoarthritis Enthesopathy (Achilles tendon) LBP c/ stiffness x >3mo

HLA-B27 Ag present in 95% of whites NSAIDs DMARDs Corticosteroid

Psoriatic Arthritis

Def:Inflamm arthritis c/ skin involvement preceding jt dz by mo-yrsMay resemble RA in clinical presentation but look at labs

Oligoarthritis, Polyarticular DIP involvement Onycholysis Nail pitting “Sausage-finger” appearance

Pathognomonic: Pencil-in-cup hand XR

↑ESR Anemia: normocytic, normochromic RF nL

NSAIDs Methotrexate → arthritis + skin

inflamm DMARDs Reconstructive surg → painful en-stage

antrhopathy

Reactive Arthritis(Reiter’s Syndrome)

Arthritis following GI or GU infection

Asymmetric arthritis MCC nontraumatic monoarthritis Often seqeulae to Ct infn

Tetrad Urethritis Conjunctivitis Oligoarthriris (knee, ankle) Mucosal ulcers (balanitis, stomatitis)

50-80% (+) HLA-B27 (-) synovial fluid cx XR/CT→ permanent/progressive jt dz

1L: PT + NSAIDs Abx @ time to infn to ↓ chance of dev

Reactive Arthritis

Enteropathic Arthritis

Arthritis associated c/ Crohn’s disease or ulcerative colitis

Nonerpsive polyarthritis of large jts Gut inflamm

Stool cx Colonoscopy if GI sx persist (-) HLA-B27

Avoid NSAIDs (may exacerbate GI sx)

Corticosteroids

5. List the historical questions and physical exam findings consistent with fibromyalgia. Refer to Table Below6. Cite the criteria recommended by the American College of Rheumatology for diagnosing fibromyalgia. Refer to Table Below7. Develop a differential diagnosis for fibromyalgia. Refer to Table Below8. Explain the pharmacologic and nonpharmacologic aspects of management for fibromyalgia. Refer to Table Below9. Describe the clinical manifestations of chronic fatigue syndrome. Refer to Table Below10. Explain the pharmacologic and nonpharmacologic aspects of management for chronic fatigue syndrome. Refer to Table Below

Fibromyalgia & Chronic Fatigue SyndromeDisorder Features SSX DX TX/PX

Fibromyalgia(FM)

DDX Pn above & below waist bilat & axial x ≥3mo

Non-articular MSK ache, pn, fatigue, sleep disturbance, multiple tender “trigger” points on PE

Pn an

Dx of exclusion

ACR Criteria1) Pn & sx > 1wk, # of painful areas out of

19 parts of body + fatigue/waking unrefreshed/cognitive sx

2) Sx x ≥ 3mo3) R/O other cause

Non-Rx Aerobic exercise, CBT, Pt edu, strength

training, acupuncture, biofeedback, balneotherapy (warm H2O therapy), hypntherapy

Rx LD TCA (for sleep), SNRI (duloxetine) Analgesics (Tramadol best) AEDs (pregabalin)

SLE Systemic ssx, ANA+

Inflamm myositis Prox mm weakness, little pn, ↑CK

Polymyalgia Rheumatica

Shldr, hip pn, elderly, ↑ESR, resp to steroids

HoT4 AbnL TFTs

Chronic Fatigue Syndrome

(CFS)

Idiopathic chronic fatigue Abrupt onset of fatigue c/ significant impairment of ADL, work ability, social relationships

Often preceded c/ flu-like prodrome Generalized MSK pn, cog dysfxn, IBS,

sleep disturbances

NOT a dx of exclusion (unlike FM)PE: (-) PE findings (unlike FM) Exclude pt c/ fatiguing med d/o,

depression, d/o, substance abuse, sev Ψobesity

Monoarthritis Oligoarthritis Polyarthritis Aches & Pains Gout/pseudogout Infectious arthritis Reactive arthritis OA Trauma

OA Psoriatic arthritis Reactive arthritis Gout/pseudoarthritis RA

RA OA Psoriatic arthritis Pseudogout SLE Gonococcal arthritis

Fibromyalgia Polymyalgia Rheumatica HoT4 Multiple soft tissue problems Hyperextensibility syndrome

∴ therefore, ∵ because, c/ with, s/ without, MCC: Most Common Cause 4