Rheumatology Basics

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Rheumatology Basics

• Kalyani McCullough, MD
• Clinics and MOP Didactic

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Purpose of this talk

• To provide a foundation for
• Seeing patients in Rheumatology clinic
• Getting started on the work-up of suspected
  rheumatologic diseases in your clinic
• Review most common rheumatologic diseases
• Fast overview
• Resource for later

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Main diseases youll be seeing

• Inflammatory arthritis
• Rheumatoid, spondyloarthropathies
• Crystal arthritis
• Gout, Pseudogout
• Autoimmune disorders
• Lupus, Sclerodema, Autoimmune myositis
• Vasculitis
• Large, medium, small vessel

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What are some clues your patient may have a
rheumatologic disease?

• Constitutional symptoms
• Fevers, sweats, weight loss, fatigue
• Subacute time course
• Started over weeks to months
• Characteristic symptoms
• Mono or polyarticular joint pain, multisystem
  complaints (i.e. skin, lung, GI)
• Are there signs of an inflammatory process?
• Synovitis, rash, serositis, elevated ESR/CRP

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How do you get started on the work-up?

• Thorough physical exam
• Lab tests
• CBC w diff, ESR, CRP, ANA, targeted
  autoantibodies
• Radiographs
• Erosive arthritis, sacroillitis
• Biopsy?
• Skin, muscle, kidney

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Basics of treatment

• NSAIDs
• Symptom relief, especially joint pain
• Glucocorticoids
• Acutely, try to use steroid sparing agent if
  possible
• Disease-modifying antirheumatic drugs (DMARDs)
• Synthetic DMARDs
• Slow onset, 2-6 months
• Methotrexate, Hydroxychloroquine, Sulfasalazine,
  Azathioprine, Mycophenolate mofetil,
  Cyclophosphamide
• Biologic DMARDs
• Bioengineered protein drugs (antibodies or
  receptor-antibody chimeras )
• IV or SQ only
• Rapid onset
• Screen for and treat LTBI, HBV first
• Infliximab, Etancercept, Rituximab

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Steroid Side Effects

• Weight gain
• Diabetes
• Cataracts
• Insomnia
• Fluid retention
• Hypertension
• Proximal weakness
• Alopecia
• Sweats
• Osteoporosis
• Infection
• Psychiatric disturbance (eg, depression, mania,
  psychosis)
• Easy bruising of the skin
• Stress
• Tremor
• Peptic ulcer disease

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Long term considerations for most autoimmune
diseases

• Increased risk of CV death due to inflammation
  and accelerated atherosclerosis
• Aggressive risk factor reduction BPlt120/80,
  LDLlt100
• Osteoporosis from glucocorticoids
• Dexa screening, Calcium-Vit D, bisphosphonates
  prn
• Infections
• Increased risk of certain malignancies
• Lymphoma, Lung/Cervical CA in Lupus

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Inflammatory Arthritis

• Rheumatoid arthritis
• Spondyloarthropathies
• Axial skeletal involvement and polyarthritis of
  peripheral joints
• Ankylosing spondylitis
• Reactive arthritis
• Psoriatic arthritis
• IBD-associated arthritis

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Rheumatoid arthritis

• Clinical presentation
• Chronic, symmetric polyarthritis
• Often starts with the PIPs, MCPs, MTPs
• Can affect most synovial joints (wrists, knees,
  elbows etc.)
• Doesnt affect DIPs
• Morning stiffness, pain, swelling (vs. OA worse
  with activity)
• Labs
• RF, anti-CCP Ab or both in 70 of patients
• RF not specific, anti-CCP 90-98 specific
• ACD, ESR, CRP
• Xrays
• articular erosions and joint space narrowing

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Joint distribution in RA vs. OA
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RA Physical Exam Findings

• Ulnar deviation
• at mcps
• Swan neck deformity
• hyperextension of PIPs, flexion of DIPs
• Boutonniere deformity
• flexion of PIP, hyperextension of DIP

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OA Physical Exam Findings
Bony outgrowths/ calcific spurs on PIPs and DIPs
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RA Extra-articular Manifestations

• Many extra-articular findings
• Rheumatoid nodules
• Other organ involvement
• Sjogrens syndrome
• Scleritis
• Interstitial fibrosis
• Pericardial effusions
• Peripheral neuropathy
• Amyloidosis

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RA - Treatment

• Early aggressive treatment leads to better
  longterm outcomes
• Glucocorticoids acutely
• MTX frequently used
• Start 7.5mg weekly, increase to a max dose of
  20-25mg weekly
• Give with folate 1mg daily to reduce toxicity
• Check CBC, AST/ALT, Cr q 3 mo
• Contraindications liver disease, HBV/HCV,
  alcohol use, CrCllt30
• Toxicities oral ulcers, nausea, hepatotoxicity,
  BMS, pneumonitis

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Sponydloarthropathies

• Inflammatory arthritis with involvement of
• Sacroiliac (SI) joint
• Axial spine
• Tendon fascia
• Ligament insertion points (entheses)
• Peripheral joints

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Spondyloarthropathies

• Ankylosing Spondylitis
• Psoriatic Arthritis
• DIPs often involved, assoc with nail pitting
• Reactive Arthritis
• Triggered by GI/GU infection
• IBD-associated arthritis
• Correlates with bowel activity

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Ankylosing Spondylitis

• Clinical presentation
• Inflammatory back pain in young adult
• Reduction in spinal mobility, particularly lumbar
  flexion
• Association with anterior uveitis
• Labs
• Increased relative risk of HLA-B27

• Xray
• - Sacroiliitis
• Treatment
• - PT, NSAIDs

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Crystal Arthritis

• Gout Natural History
• 1) Asymptomatic hyperuricemia
• Over production or under excretion (or both)
• 2) Acute and intermittent gout
• Attacks triggered by fluctuations in UA level (up
  or down) and deposition of UA crystals in joint
• Acute monoarthritis, often 1st attack is at 1st
  mtp
• After years see bony erosions on xray
• 3) Chronic tophaceous gout

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Gout - Treatment

• Acute attack most efficacious if initiate
  treatment within 48h of onset
• NSAIDs indomethacin
• Avoid if h/o PUD or renal impairment
• Colchisine
• 1.2mg PO x 1, then 0.6mg 1h later and q24h until
  symptoms improve
• Side effects Diarrhea, abdominal pain, NSAIDs
  better tolerated
• Reduce dose if Cr gt 1.5, risk of neuromuscular
  toxicity
• Glucocorticoids
• Prednisone 20-40mg/d, taper 1-2 wks after
  symptoms resolve

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Gout - Prophylaxis

• Goal UAlt6
• Xanthine oxidase inhibitors (decrease production)
• Allopurinol
• Start with 100mg daily, gradually increase dose
• Max is 800mg/d, though 300-400mg usually
  effective
• Reduce dose if diminished Cr
• Hypersensitivity reaction can be fatal, more
  common in older patients with impaired Cr on
  diuretics
• If develop rash, stop
• Febuxostat
• Metabolized by liver, so better with renal
  impairment
• Ok with mild-moderate hepatic insufficiency
• Uricosuric agents (increase excretion)
• Probenacid
• Age lt65, good renal function, not on more than
  ASA 81 daily
• Side effects rash and GI symptoms

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Pseudogout

• Clinical presentation
• Intermittent monoarthritis, often knee/wrist,
  though can be 1st mtp
• Labs
• rhomboid shaped, positively birefringent calcium
  pyrophosphate dihydrate (CPPD) crystals
• Can be associated with other diseases
  hemochromatosis, thyroid disease
• Treatment
• NSAIDs, colchisine

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Autoimmune disorders

• Systemic lupus erythematosis (SLE)
• Antiphospholipid antibody syndrome
• Raynauds phenomenon
• Scleroderma
• Sjogrens Disease
• Dermatomyositis and Polymyositis
• Relapsing Polychondritis

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Lupus

• Clinical presentation
• Typically affects women of childbearing age
• Multisystem relapsing/remitting disease
• Affects skin, musculoskeletal, renal, CV,
  pulmonary, GI, CNS, psych

- Hallmark symptoms photosensitive rash,
polyarthritis, serositis, fatigue - Renal and
CNS involvement -gt significant morbidity
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Lupus

• Labs
• Presence of antinuclear antibodies
• Once ANA positive, no need to recheck
• Anti-dsDNA and anti-Sm have great specificity,
  but lack sensitivity.
• Hypocomplementemia may occur during flares.
• C3, C4, CH50
• Treatment
• Glucocorticoids, DMARDs

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Lupus - Monitoring

• Lupus nephritis approx 50
• Typically immune complex glomerulonephritis
• Screen q3 mo
• Ask about polyuria, nocturia, foamy urine
• Look for HTN and edema
• Labs UA RBC casts, spot prot Cr ratio
• Diagnose with biopsy
• Increased risk of malignancy Hodkins and
  Non-Hodgkins lymphoma, Lung CA, Cervical CA
• Annual cervical CA screening

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Scleroderma

• Clinical presentation
• Early in disease
• msk pain, fatigue, weight loss, GERD
• Rare disorder, but symptoms common in primary
  care
• Late in disease
• Skin thickening, ischemic digital ulcers,
  pitting, telangectasias on face and palms
• Lungs interstitial fibrosis, pulmonary htn
• GI GERD, dysphagia
• Renal rare, but Scleroderma Renal Crisis (SRC)
  is life threatening emergency
• Cardiac ischemic injury
• MSK erosive arthritis, tendon friction rub
• CREST (limited scleroderma) calcinosis,
  raynauds, esophageal dysmotility, sclerodactyly,
  telangectasias

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Scleroderma
Thickened, tight skin
Digital ulcers
Sclerodactyly
Telangectasias
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Scleroderma

• Labs
• If negative ANA, diagnosis is very unlikely
• Indirect immunofluorescence better than ELISA
• Anti-centromere Ab in 20-40
• Anti-Scl-70 (anti-topoisomerase) 20-40
• Correlates with worse prognosis
• Treatment
• No proven disease-modifying medication
• Screening and early identification of internal
  organ involvement important
• Organ specific treatment
• Fibrosis immunosuppresion
• Renal crisis ACEi
• GERD PPI and behavioral change

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Autoimmune myositis

• Polymyositis (PM)
• Dermatomyositis
• Dermatomyositis sine myositis (just skin)
• Dermatomyositis sine dermatitis (classic muscle
  biopsy findings)
• Immune-mediated necrotizing myopathy (IMNM)
• Statin exposure

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Autoimmune myositis

• Clinical presentation
• Symmetric, proximal muscle weakness that develops
  over weeks to months
• Difficulty rising from a chair, walking up steps,
  washing or brushing hair
• If severe dysphagia and respiratory distress
  from weakness of neck, pharyngeal and
  diapghragmatic muscles
• Distal weakness very uncommon, suspect another
  diagnosis

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Dermatomyositis

• Clinical Presentation Skin
• Gottron papules raised violaceous lesions at
  extensor surfaces of mcp, pip, dip
• Gottron sign erythematous rash at above sites
• Heliotrope rash red/purplish discoloration of
  eyelids

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Dermatomyositis

• Shawl sign erythematous rash across posterior
  neck and shoulders
• V sign erythematous rash on anterior neck and
  chest
• Mechanics hands/feet hyperkeratotic skin
  thickening, often with painful cracking

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Autoimmune myositis

• Labs
• Elevated CK, aldolase, AST, ALT, LD from damaged
  muscle cells
• Auto antibodies (anti-Jo-1, anti PL-7)
• ANA positive in gt50
• Antisynthetase syndrome in dermatomysoitis also
  with ILD, arthritis, fevers, raynauds
• MRI muscle edema, fascial inflammation
• EMG irritable myopathy
• Muscle biopsy generally nonspecific findings,
  but rules out non-autoimmune myopathies
• DM perifascicular atrophy is specific

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Autoimmune myositis

• Treatment
• Glucocorticoids to control acute disease
• Taper to lowest effective dose
• Steroid sparing agent MTX, azathioprine
• Exercise especially isometric, avoid heavy
  weight lifting

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Autoimmune myositis vs. PMR

• Polymyalgia Rheumatica (PMR)
• aching and stiffness of neck, shoulders, hips
  (vs. weakness)
• Elevated ESR gt40
• Age gt50
• Rapid response to prednisone 20mg/d or less
• Association with GCA, but can occur independently
  too

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Mixed Connective Tissue Disease

• Overlap syndrome with features of SLE,
  Scleroderma and Polymyositis
• Characteristic clinical symptoms
• Raynaud phenomenon, hand edema, synovitis,
  inflammatory muscle disease, sclerodactyly
• High titers of anti-U1 RNP autoantibody

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Vasculitis

• Inflammatory destruction of blood vessels
• Occlusion, rupture, thrombus can lead to tissue
  ischemia
• Vary in severity and presentation based on
  vessels affected

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Vasculitis

• Clinical presentation
• Constitutional symptoms
• Subacute
• Signs of inflammation
• Multisystem disease
• i.e. Mononeuritis multiplex
• Distinct/named peripheral nerves infarcted one at
  a time
• Due to vasculitis of vasa nervorum leading to
  ischemia
• Hallmarks are asynchrony and asymmetry
• Labs
• Diagnose with biopsy
• Treatment
• Disease specific, often immunosuppressive therapy

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In Summary

• Suspect a rheumatologic process in patients with
  subacute onset of constitutional symptoms, signs
  of inflammation and multi system involvement
• Get work-up started with thorough PE, targeted
  labs and xrays
• Consult with Rheum and consider getting
  prednisone started to control acute disease, with
  a goal of tapering to the lowest effective dose
  when possible
• Monitor for the longterm complications of
  autoimmune disease

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Questions?