72
Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology.

Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Embed Size (px)

Citation preview

Page 1: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Rheumatology Board Review Presentation - 2

Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology.

Page 2: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Game-Plan

Stimulate your mind and think with me

Highlight the topicsGo over “educational objectives” These are potential questions/test

pertinent areasParticipate If you want write, Use the handout

from today otherwise just listen

Page 3: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

SUBJECTIVE COMPLAINTS OF WEAKN ESS

AS YMMETRIC Regional neuro logical disorders Cerebrovascular disorders Spinal cord and spinal root disorders Demyelinating disease Compression neuropathy Mononeuropathy/mononeuritis multip lex Disuse atrophy Myasthenia gravis

SYMMETRIC

SPECIFIC PATTERN Muscular dystrophy Hereditary neuropathies Myasthenia gravis

PROXIMAL Inflammatory myopathy Metabolic myopathy Endocrine myopathy Glucocorticoid myopathy Mitochondrial myopathy Drug-induced myopathy HIV-related myopathy Duchene’s muscular dystrophy Myasthenia gravis

DISTAL Peripheral polyneuropathy Motor neuron disease Myasthenia gravis

Exclude: Anemia Card iopulmonary disease Chronic infections Malignancy Depression Fibromyalgia Malingering Deconditioning

OBJECTIVE MUS CLE WEAKNESS

Inflammatory Myopathy: • Symmetric proximal muscle weakness • Elevated plasma muscle enzymes • Myopathic changes on electromyography • Characteristic muscle biopsy abnormalities and the absence of

histopathologic signs of other myopathies • Typical rash of dermatomyositis

Management includes complete laboratory profile, EMG, Biopsy. Work-up for underlying malignancy. Start prednisone at .5 mg/kg- 1 mg/kg initia lly. Needs appointment in rheumatology in 4-6 weeks or earlier.

Page 4: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Dermatomyositis-PolymyositisClassification CriteriaFive criteria for DM and PM were proposed in

1975 Symmetric proximal muscle weakness Elevated plasma muscle enzymes Myopathic changes on electromyography Characteristic muscle biopsy

abnormalities and the absence of histopathologic signs of other myopathies

Typical rash of dermatomyositis 

 

Page 5: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Dermatomyositis-PolymyositisOther clinical features1. fever and weight loss, 2. A nonerosive inflammatory polyarthritis, 3. Raynaud's phenomenon 4. Cardiopulmonary abnormalities also may

occur. 5. Congestive heart failure due to myocarditis6. Interstitial lung disease

MYOSITIS SPECIFIC ANTIBODIES:Anti T Rna Synthetases : Anti Jo-1: associated

with ILDAnti Mi-2 (Dm)Anti Pm-SCLAnti SRP: associated with cardiac disease

Page 6: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Heliotrope Rash

Page 7: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

©Copyright Science Press Internet Services

Gottron’s sign/papules

Page 8: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology
Page 9: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Clinically, DM is associated with certain skin manifestations.

Ocular muscles are more frequently involved in DM.

DM Has a higher incidence of malignancy, although various cancers (GI, Lung, Ovary) can be associated with both DM and PM.

Pathogenetically, DM is associated with immune complex deposition in the vessels, whereas PM appears to reflect direct T cell-mediated muscle injury.

DIFFERENCES BETWEEN DM AND PM

Page 10: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

TREATMENT

Corticosteroids 1 mg/kg are medications of choiceIV pulse steroids may be neededUsually needed for a long duration: months to years.

Immunosuppressive therapies:

Azathioprine (Imuran)Methotrexate Combination of MTX and imuranIV cyclophosphamideIVIGBiologics- TNF blockers, Rituximab

Page 11: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology
Page 12: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Inflammatory Myopathy Questions asked1. Patient scenario: middle aged or elderly female.

Male age above 50.2. Patient presents with weakness, hand and eyelid

rash. Work-up should include? Likely diagnosis? 3. Same patient is at risk for what? What tests to do?

Additional tests in female patient?4. Patient with myositis presents with cough, SOB.

What test? Antibody? Diagnosis? 5. Patient with myositis responded to steroids and

MTX now weak again, CPK is 650. Diagnosis? Management?

6. 65 year-old-male comes with weakness, CPK 600, abnormal EMG. Treated with prednisone, MTX for 5-6 months but no response. Diagnosis? Appropriate test? Treatment?

7. Myositis patient with left hip pain and abnormal exam. What test? Likely diagnosis?

Page 13: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

MonoarthritisMonoarthralgia

Exclude Periarticular DiseaseTendonitis, Bursitis, Bone lesion, Myofascial Pain, Sprain/Overuse, soft tissue infection, peri-articular GC infection

Monarticular Arthritis/ ArthalgiaAlways consider Aspiration of joint for diagnosisCell count, Crystals, Gram stain, Culture

Crystalline diseaseGout/pseudo goutTrue inflammatory changes in joint with urate or CPPD crystals on synovial fluid examination; cell count of 2000-50,000; Cystic erosions (gout) or chondorcalcinosis (pseudgout) on X-ray

Osteoarthritis/traumaAbsence of inflammatory changes and <30 minutes AM stiffness with the presence of pain, crepitus, mild swelling, osteophytes; X-ray changes of joint narrowing, osteophytes formation, sclerosis; Joint fluid aspirate either bloody or cell count<2000; If diabetic, consider neuropathic joint

Seronegative spondyloarthropathyInflammatory changes of joint (particularly knee or ankle);Synovial fluid cell count of 2000- 50,000; involvement of eyes (conjunctivitis or uveitis) or back/sacroiliac joints with prolonged (>1 hour AM stiffness)

Early onset inflammatory arthritisRA or SLEInflammatory joint changes with other etiologies excluded; with prolonged (>1 hour AM stiffness); with positive RF or ANA

InfectionGonococcusStaphylococcusStreptococcusDiagnostic features: True joint Inflammation with pain, swelling, erythema, Leukocytosis and fever, Synovial fluid cell count >50,000, Positive gram stain and culture

Page 14: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Septic Arthritis

Page 15: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Synovial Fluid Analysis

Normal Inflammatory Septic Hemorrhagic

Crystal Induced

Clear/YellowTransparent

Yellow/ WhiteTranslucent/opaque

Yellow/WhiteOpaque

RedOpaque

Yellow/ White, Translucent/opaque

<2K 2K-50K >50K N/a 10K-50K

-ve Gm stain & Culture (Cx)

-ve Gm stain & Culture

+/- Gm stain, +/- Culture

-ve Gm stain, Cx.

-ve Gm stain, Cx.+ Crystals

Page 16: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Disseminated GC

Page 17: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Skin pustules in DGC

Page 18: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Infectious arthritisQuestions asked1. Patient scenario: Male, Acute

symptoms (sub-acute female in DGI)2. Elderly male presents to the ER with

acute R knee pain/swelling: Next test, Results abnormal- What to do?

3. 20-40 year old F with migratory arthritis: Knee-ankle-wrist, Tap shows 15,000 wbc. Has a rash. Diagnosis?

4. Travel to Connecticut, rash, fatigue, myalgia? History? Diagnosis? Treatment?

5. Same patient (did not get treatment)1 year later has CN palsy/knee arthritis. What to do?

Page 19: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

InflammatoryAM stiffness > 1 hourJoint erythema, swelling, or pain

AndElevated ESR (must exclude other inflammatory diseases such as infection or cancer)

OrPositive RFPositive ANAImplies a differential diagnosis of:Rheumatoid arthritisConnective tissue disease (lupus, PSS, PM/DM, Sjogren’s)Viral infectionSeronegative spondyloarthropathySarcoidosis

Non- InflammatoryAM stiffness < 1 hourNo joint erythema, swelling andNormal ESRNegative RF or ANAImplies a differential diagnosis ofMyofascial painFibromyalgiaOsteoarthritisEndocrinopathyPain syndrome

Does not clearly fit into either category, Consider:Seronegative RASeronegative spondyloarthropathy

Polyarthritis/ Polyarthralgia

Determine by History and Physical if inflammatory(Prolonged AM stiffness, erythema, swelling, warmth, pain)

Evaluation dictated by history and physical may include CBC, urinalysis, liver and kidney function, RF, ANA, ANCA, ESR, TSH, CPK, HBA1C, CXR and regional/back/SI joint X-rays

Page 20: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

SPONDYLOARTHROPATHIES

Ankylosing spondylitisReactive Arthritis – (Reiter’s)Arthropathy of inflammatory bowel

disease (Enteropathic Arthritis) Psoriatic arthritisUndifferentiated spondyloarthropathiesJuvenile chronic arthritis and juvenile-

onset ankylosing spondylitis

Page 21: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

CLINICAL CHARACTERISTICS OF SPONDYLOARTHROPATHIES Tendency to cause sacroiliitis Tendency to cause spondylitis Association with enthesopathy (enthesitis) Typical pattern of peripheral arthritis: usually

lower limb, asymmetric, tenosynovitis (sausage digits)

Absence of rheumatoid factor (RF) Absence of subcutaneous nodules and other

extra-articular features of rheumatoid arthritis (RA)

Overlapping extra-articular features characteristic of the group (e.g., anterior uveitis)

Significant familial aggregation and association with HLA-B27

Page 22: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Sausage Digits

Page 23: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Management

Physical modalitiesNSAIDS - 1st line (Mild cases/stable

dz-only Rx) DMARDs (? Efficacy – SSZ, others)New treatments- TNF alpha blockers.

Page 24: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Inflammatory back painHLA B-27 associated Symmetrical SacroilitisSpondylitis: erosion, squaring of the

vertebraeUveitis; symmetricalAtypical presentations: female, older

ageOcciput to wall testSchobere’s testEnthesopathy.

Ankylosing Spondylitis

Page 25: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Inflammatory oligoarticular arthritis in the setting of previous or intercurrent nongonococcal urethritis and/or conjunctivitis is characteristic of patients with Reiter’s syndrome.

It is important to recognize that the majority of patients with this disorder present with just one or two features of the triad.

Clinical triad of urethritis, conjunctivitis, and inflammatory arthritis.

Mucocutaneous manifestations of Reiter’s syndrome: Circinate Balanitis and keratoderma

Enthesopathy. Remember- Reactive arthritis of GI origin,

Enteropathic arthritis & PsA

Reactive Arthritis - GU (Prev Reiter’s Syndrome)

Page 26: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Keratoderma Blenorrhagica

Page 27: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Spondyloarthropathy/Infectious arthritisQuestions asked1. Patient scenario: Male, sub-acute symptoms 2. Patient with back pain worse in AM and

better with activity. What test? What eye manifestation (different in RA)? Differentiate from non-inflammatory back pain?

3. AS patient come with widened pulse pressure, heart murmur. What test? Diagnosis?

4. AS patient comes with weakness in legs, BB incontinence. Diagnosis? Treatment?

5. AS patient with pain/numbness affecting all 4 Ext UE>LE? Recently rear-ended? Next test? Diagnosis?

6. AS patient with SOB? Diagnosis?

Page 28: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Spondyloarthropathy/Infectious arthritisQuestions asked7. Patient with R knee arthritis, h/o STD.

Diagnosis? Eye manifestation? Associated Disease?

8. Patient with painful DIP, swollen 2nd toe. Exam has nail pitting, What else to look for? Diagnosis?

9. Japanese patient with recurrent oral/genital ulcers, arthritis. Diagnosis? Treatment?

10. Cruise ship (trip to Mexico etc) had diarrhoea, Now with 1-2 joint arthritis +/- sausage toe. Likely diagnosis? Associated organisms?

11. AS patient with back pain not responding to NSAIDs and SSZ. Treatment?

Page 29: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

THE VASCULITIDES

CLASSIFICATIONSIZE OF THE VESSELS INVOLVED ORGAN SYSTEMS INVOLVED

Page 30: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Large vessel: GCA and temporal arteritis, Takayasu’s arteritis, Primary angitis of CNS

Medium size vessels: PAN, Kawasaki disease, Granulomatosis with polyangitis i.e. GPA (Prev Wegener’s granulomatosus) EGPA (Prev Churg strauss syndrome),

Small vessels: GPA (Wegener’s), EGPA (Churg Strauss), Henoch Schonlein Purpura, Microscopic polyangitis (MPA), Mixed cryoglobulinemia, Hypersensitivity vasculitis/leukocytoclastic vasculitis, Vasculitis associated with rheumatic diseases- RA,SLE

Misc. : Behcet’s disease, Relapsing polychondritis, Lymphomatoid granulomatosis

CLASSIFICATION

Page 31: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

CLINICAL MANIFESTATIONS NON SPECIFIC:Fatigue and weaknessFever, skin rashArthralgias and/or arthritisabdominal pain hypertension neurological dysfunction/neuritis. SPECIFIC: PERTAINING TO THE ORANS INVOLVED Pulmonary nodulesSinus /nasal inflammationGastrointestinal inflammation with its presenting symptomsGlomerulonephritis/renal insufficiency with an active

sediment.

Page 32: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Hypersensitivity /leukocytoclastic vasculitis

Age >16 a possible offending agent in temporal relation to

the symptoms Palpable purpura Maculopapular rash Biopsy of a skin lesion showing neutrophils around

an arteriole or venule

ANY OF THE VASCULITIDES WHICH CAN INVOLVE SMALL VESSELS CAN PRESENT AS PALPABLE PURPURA & HAVE A PATHOLOGY OF LEUKOCYTOCLASTIC VASCULITIS

Page 33: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

THERAPY Hypersensitivity vasculitis –

• Discontinuing the offending agent may be adequate

• NSAIDS,Antihistamines.• Colchicine, dapsone• Corticosteroids as needed• Occasionally cytotoxic agents are required.

Systemic vasculitis :• Corticosteroids are almost always needed.• Cytotoxic agents have to be used in a number of

instances.• Rituximab in ANCA associated Vasculitis (AAV)

Page 34: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

                                                                                   

Page 35: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Classification Criteria GPA – Wegner’s Granulomatosis

Nasal or oral inflammation (painful or painless oral ulcers or purulent or bloody nasal discharge)

Abnormal chest radiograph showing nodules, fixed infiltrates, or cavities

Abnormal urinary sediment (microscopic hematuria with or without red cell casts)

Granulomatous inflammation on biopsy of an artery or perivascular area

C-ANCA (anti proteinase-3) is now an important diagnostic test.

Up to 90% sensitive in active systemic disease.

Tissue biopsy can be diagnostic--A nasopharyngeal lesion, Renal biopsy, Lung biopsy

Page 36: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Saddle nose

Page 37: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Nodules

Page 38: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

POLYARTERITIS NODOSAACR Classification criteria: 3/10

+ are 82% sensitive, 84% specific

• Otherwise unexplained weight loss greater than 4 kg • Livedo reticularis • Testicular pain or tenderness • Myalgias (excluding shoulder and hip girdle), weakness of

muscles, tenderness of leg muscles • Mononeuropathy (Mononeuritis Multiplex) or

polyneuropathy • New onset diastolic blood pressure greater than 90 mmHg • Elevated levels of serum blood urea nitrogen or creatinine • Evidence of hepatitis B virus infection • Characteristic arteriographic abnormalities • A biopsy of small or medium-sized artery containing PMN’s.

Page 39: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Renal angiogram

Page 40: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

PMR is classically characterized by aching and morning stiffness in the shoulder, hip girdles, neck and torso in patients over the age of 50.

The symptoms are usually symmetric and associated with a high ESR, but asymmetric pain and normal ESR can occur.

GCA (temporal) arteritis is a chronic vasculitis of large and medium size vessels which commonly occurs among individuals over 50 years of age.

Polymyalgia rheumatica occurs in about 50 percent of patients with GCA, while approximately 15 percent of patients with PMR as the primary diagnosis develop GCA

Giant Cell (Temporal) Arteritis & Polymyalgia Rheumatica

Page 41: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Symptoms/signs• NON SPECIFIC:fatigue, weakness, fever, arthralgias.

• SPECIFIC: new headache(60-70 %) jaw claudication (30-40%)Permanent partial or complete loss of vision in

one or both eyes(15-20%)arm claudication (10 %) Tender or thickened temporal arteries.Bruits in cranial and upper extremity vessels

• Characteristic laboratory abnormality is a high ESR upto 100mm/h. normal values may occasionally be seen.

Page 42: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology
Page 43: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology
Page 44: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

CRITERIA FOR GCA

Age greater than or equal to 50 years at time of disease onset

Localized headache of new onset Tenderness or decreased pulse of the

temporal artery Erythrocyte sedimentation rate greater than

50 mm/h (Westergren) Biopsy which includes an artery, and reveals a

necrotizing arteritis with a predominance of mononuclear cells or a granulomatous process with multinucleated giant cells

Page 45: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

TREATMENTCorticosteroids are the mainstay of treatmentPrednisone is started in at 10-20 mg for PMR

and titrated to a response in clinical and laboratory markers.

For GCA prednisone is started at a higher dose of 0.5 to 1 mg/kg. IV steroids may be needed for acute visual changes. Subsequently Slow taper is recommended.

Usually treatment is continued for 1- 2 years after remission.

Cytotoxic medications and methotrexate can be used and may be required both during acute and chronic phases.

Page 46: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Classification Criteria for Relapsing Polychondritis• Recurrent chondritis of both auricles• Nonerosive inflammatory polyarthritis• Chondritis of nasal cartilage• Inflammation of ocular structures,

including conjunctivitis, keratitis,

scleritis/episcleritis and/or uveitis• Chondritis of the respiratory tract

involving laryngeal and/or tracheal cartilages

• Cochlear and/or vestibular damage manifest by neurosensory hearing loss, tinnitus, and/or vertigo

Page 47: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

The VasculitidesQuestions asked1. Elderly (>50) patient presents with fever,

stiffness? Next test? Treatment?2. Same patient has headache, visual loss. Next

test? Shortcoming of the test? Treatment?3. Young Asian/Hispanic patient with pain,

weakness in left arm. Finding on exam? Next test? Diagnosis?

4. Male patient with abdominal pain, weight loss, arthritis, leg ulcers, purpuric rash. Next test? Diagnosis? Associated findings?

5. Same patient also has history of IV drug abuse. underlying disease? What results on ANCA testing?

6. Same patient with foot drop. Diagnosis? Next test?

7. 40-50 year old male patient with abdominal pain, arthritis, leg ulcers, purpuric rash. Next test? Diagnosis? Associated findings?

Page 48: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

The VasculitidesQuestions asked8. Male patient with SOB, cough, weight loss, active

urinary sediment, + p-ANCA. Next test? Diagnosis? Treatment?

9. Patient with sinusitis, nasal ulcer. Next test? Diagnosis? Treatment?

10. Same patient with hemoptysis, cough, renal insufficiency? Next test? Diagnosis? Treatment?

11. Patient with h/o asthma, rhinitis, now has cough, purpuric rash. chest x-ray: infiltrates. Next test? Diagnosis? Treatment?

12. Purpuric rash, URI, hematuria. Diagnosis? Treatment?13. Urticarial rash, hematuria- active urinary sediment-

Dx & Tx?14. Recreational drug assoc pseudovasculitis- clinically

vasculitis, -ve imaging & bx. Serology- ANCAs may be +. Culprit drugs? Cutting agents? Tests?

15. Patient started on HCTZ, now has a rash: palpable purpura. Diagnosis? Management?

16. Patient of Mediterranean descent with recurrent abdominal pain, fever, arthritis. + family history. Diagnosis? Treatment? Complication?

Page 49: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Osteoporosis: Diagnosisand Evaluation

Central DXA (Dual Energy X-ray Absorptiometry) remains the state-of-the-art diagnostic standard

Bone density is the most important predictor offracture risk

Page 50: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology
Page 51: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

What is a T-score? What is a Z-score?

The BMD results are expressed as standard deviations from a comparison to the referent mean.

A T-SCORE is the number of standard deviations the bone mineral density measurement is above or below the YOUNG-NORMAL MEAN bone mineral density.

A Z-SCORE is the number of standard deviations the measurement is above or below the AGE-MATCHED MEAN bone mineral density.

Page 52: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

World Health Organization Interpretation of Bone Density

Patient’s T-Score

-0.5 T

-1.0 T

-1.5 T

-2.0 T

-2.5 T

Classification

Normal

Osteopenia

OsteoporosisSevere Osteoporosis(with fragility fractures)

Page 53: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

National Osteoporosis Foundation

Who should be evaluated for OP.

1. Risk factors for osteoporotic fracture include: low body weight; history of fracture; Caucasian; cigarette smoking; estrogen deficiency, early menopause; low calcium intake; alcoholism; recurrent falls; and inadequate physical activity.

* Not well-defined

• Bone density test vs Fracture risk assessment?

• Women: o Age > 65 without risk factorso Age > 50 with risk factors1

• Meno Age > 70o Earlier – if there are risk factors*- osteoporotic fracture, hypogonadism, malignancy etc.

Page 54: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Fracture Risk Assessment Score- FRAX

• FRAX- Tool to assess fracture risk (Developed by WHO) • Decision making alogrithm for assessing the risk of fracture in an individual based on clinical information and using that to make therapeutic decisions.

• Can add BMD in the calculation

• Gives a 10-year risk

• A 10 year risk of overall fracture > 20% or hip fracture > 3% is felt to be significant.

Page 55: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

National Osteoporosis Foundation

Who should be considered for treatment of OP.

• Complicated answer

• Women: o Age > 40 with low BMD (T-score <-2.5 i.e. OP) or high FRAX (T-score>1&<2.5 i.e. osteopenia)o Age < 40- individualize (risk factors, fracture, steroid use)

• Meno low BMD (T-score <-2.5) or high FRAXo Younger patient, ?Age - individualize (risk factors, fracture, steroid use)

Page 56: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

*800 IU/day for patients over age 65

Traditional Nonpharmacological Management for Prevention and Treatment

Calcium (1200 – 1500 mg/day individed doses)

Vitamin D 400-800 IU/day*Weight-bearing exerciseFall-prevention techniques

Page 57: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Fracture Prevention: Role of Drug Therapy Anti-resorptive therapies:Hormone replacement therapy (HRT)RaloxifeneBisphosphonates (C/I if GFR < 30)

Alendronate Risedronate Ibandronate Pamidronate Zolendronate

CalcitoninRANK Ligand inhibitor- Denosumab (Prolia) (OK in

moderate/sever renal disease)

Bone formative: PTH (Forteo)

Page 58: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Osteoarthritis Primarily a disorder of cartilage associated with

cartilage destruction and secondary bone changes Most Common rheumatic disorder Common cause of joint pain especially in elderly Joint Pain: weight bearing joints, worse with activity

& better with rest AM stiffness is minimal Typically involves DIP and PIP Can have secondary synovitis (inflammation) Common cause of back pain: remember SPINAL

STENOSIS and its features Typically normal lab studies X-rays: joint space narrowing and osteophyte

formation

Page 59: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology
Page 60: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology
Page 61: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

OsteoarthritisManagementManagement

•AcetaminophenAcetaminophen•NSAIDs: Any NSAID works fineNSAIDs: Any NSAID works fine•Weight reduction and physical therapyWeight reduction and physical therapy•Local therapyLocal therapy•Intra-articular steroids Intra-articular steroids •Intra-articularIntra-articular viscous supplementation ( Hyalgan, Synvisc etc) viscous supplementation ( Hyalgan, Synvisc etc) only in knee OAonly in knee OA•NarcoticsNarcotics

Role of joint replacement Surgery:Role of joint replacement Surgery:•Treat patient not radiographTreat patient not radiograph•Symptoms of Rest pain and night pain are indications for surgerySymptoms of Rest pain and night pain are indications for surgery•Functional impairment is also indication for surgeryFunctional impairment is also indication for surgery•Adequate medical work-up due to risk factorsAdequate medical work-up due to risk factors

Page 62: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Back Pain Mechanical Back PainMinimal AM stiffnessIntermittent Improved with restDecreased ROM in all directions

Inflammatory Back painProlonged AM stiffnessPersistent Improved with activityDecrease in Flexion/extension of spine: Schobere’s test

Remember symptoms and neurological signs of nerve impingement syndromes at various levels

Remember back pain (typically at night time) in elderly could be sign of malignancy

Remember back pain in elderly/immunosuppresed could be sign of chronic infection typically TB

Page 63: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Fibromyalgia Clinical Syndrome not a disease Typically middle aged white females No objective sign of joint inflammation Associated with myofascial pain, fatigue,

stiffness, lethargy Alterations in normal sleep pattern Associated with psychiatric syndromes, IBS,

migraine, premenstrual syndrome No diagnostic test: must rule out a serious

underlying disorder Tender points on examination ( 11 out of 18)

Management includes: counseling, improving sleep hygiene, graded exercise, SSRIs, Muscle relaxants, Ultram, treatment of psychiatry disorders

Page 64: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Osteoarthritis, OP, Fibromyalgia and Back painQuestions asked1. Patient with severe, persistent knee/hip pain, night

pain. Treatment? 2. X-Ray findings, Treatment algorithm for OA3. 70-year-old female with back pain, night time

worsening, point tenderness. Likely Diagnosis?4. 30-year-old with back pain radiating to leg. No

neurological deficit. Diagnosis? Treatment?5. 45 year old with left sided back pain, radiation to

foot, can not dorsi-flex big toe. What level is impingement? What reflex to look for?

6. 35 year old female with fatigue, aching, poor sleep, ANA 1:40. Likely diagnosis? Treatment?

7. 50 year old with history of radius fracture? What to work-up for? Test of choice?

8. Male with OP? testing?9. Secondary causes of OP?10. Comparative efficacy of various agents, SE and C/I11. Fracture risk reduction of various agents

Page 65: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Other Board Pertinent Disorders

Pain, swelling of extremity after trauma. Area cool to touch, edematous, Hyperalgesia? Diagnosis? Test of choice?

Shoulder Injury and now pain in wrists/hands with above findings. Treatment?

Lyme disease- Arthritis (2nd stage), associated fibromyalgia. No ABX treatment of arthritis if treated in past

Pain hip: remember back/spine origin, tender outside (lateral side) of hips-diagnosis?

Pain knee: Normal exam,, Look for hip abnormality.

Pain knee: normal exam- ilio-tibial band syndrome. Medial side of tibia. Diagnosis?

Page 66: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Other Board Pertinent DisordersSarcoidosis: EN, Lofgren’s syndrome,

othersObese or diabetic patient with

pain/burning pain ant thigh. Mild Sensory loss. Normal EMG. Diagnosis?

60 year old, smoker with pain in ankles, hands. Tender above ankle, clubbing, radiographs periosteitis. Diagnosis?

Corticosteroid toxicities including associated myopathy, AVN and osteoporosis

Carpal Tunnel Syndrome & association with hypothyroidism.

Page 67: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Buerger’s disease/ Thromboangiitis obliterans

Non-atherosclerotic, segmental, inflammatory disease that most commonly affects the small and medium sized arteries, veins, and nerves of the extremities

Normal ESR & CRPCellular/Inflammatory thrombus Normal: auto-antibodies/other serologic testsCork screw blood vesselsStop smoking

Page 68: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Osteomalacia, Vitamin D deficiency

Usually, the cause of osteomalacia is a deficiency in vitamin D

There are other causes: Low phosphate

Normal 25-OH vitamin D levels: 30 ng/ml

Replace with 50, 000 units once or twice a week for 3-6 months

Page 69: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

DISH Calcification

and ossification along the anterolateral aspect of at least four contiguous vertebral bodies

Preservation of intervertebral disc height

Lack of findings of AS

Page 70: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Paget’s Disease (osteitis deformans) Focal skeletal disorder characterized by an

accelerated rate of bone turnover. CLINICAL MANIFESTATIONS Pain Skeletal deformities and fractures Bone tumors (remember osteosarcoma) Neurologic complications (Hearing loss, CN

Palsies) Cardiac complications: Heart failure, Aortic

stenosis, Conduction abnormalities Calcium and phosphate metabolism, Elevated

Alkaline phosphatase.Treatment: Bisphosphonates

Page 71: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

Erythema NodosumCauses Idiopathic Sarcoidosis IBD TB Fungal URI: Viral/Bacterial OCs CTD

Treatment NSAIDs Potassium Iodide GCs Treat underlying disease

Page 72: Rheumatology Board Review Presentation - 2 Vikas Majithia, MD, MPH. Professor of Medicine, Chief and Fellowship Program Director, Rheumatology

GOOD LUCK

(REMEMBER DINNER AT CHAR IS GOING TO AWAIT YOU)