Joints, Rheumatology, and the Shelf

Paul Johnsonprepared by Ryan Sanford

Chief Lecture

The Joints44F mother of four children ages 3-8y is evaluated for 2wk of aching in joints of wrists, hands, and knees. Pain and swelling were severe for ~ 1 week, then subsided to aching. Pain is worse in the morning and abates somewhat with activity. On PE there is tenderness with pressure on the dorsa of the wrists and pain with wrist motion. One side of the patient’s face shows faint redness. She has noticed patchy sloughing of the epidermis of her hands. What is the diagnosis?

What is the DDx for acute arthritis?

Joint PainDuration

Acute Chronic1. Infection [septic arthritis]2. Trauma/Blood3. Crystals! Gout and CPPD4. Reactive5. Parvovirus B196. Early Chronic

Inflammation?

No = OA

Activity doesn’t help

# Joints Involved

Poly1. RA = symmetric2. SLE = symmetric3. Systemic Sclerosis = symmetric

Oligo1. Spondyloarthropathy2. Indolent infection3. Early poly

Mono1. Indolent infection2. Early oligo/poly

Activity helps, stiff in AM [>1h]!

Finding?

Hand Pains• 82F w/ chronic non-inflammatory hand pain and nodules at DIP joint -- Disease and

Eponym?– OA and Heberden’s Nodes

• Pencil in cup Deformity on Hand X-Ray?– Psoriatic Arthritis, occurs at DIP, is erosive

• Ulnar Deviation?– Rheumatoid Arthritis

• Dactylitis? AKA? – Reactive Arthritis, Sickle Cell Anemia, Psoriasis, Akylosising Spondylitis, Tb

• + anti cyclic citrullinated peptide? – RA

• Nodules filled with urate over fingers?– Gout

• MCP pain and a discoid rash?– SLE

Radiographic Findings and Dx?

Osteoarthritis• On Radiographs

– Joint Space Narrowing– Subchondral Cysts– Osteophyte Formation– Subchondral Sclerosis

• The Patient Says– Not too stiff upon awakening [<30 min]– Pain gets worse with activity– Can have some effusions, esp at knees

• Tx: – OTC analgesia – APAP, NSAIDS. No Narcotics– Intra-articular injections– PT and periarticular muscle strengthening– Joint replacement

. . . And I have pain with deep breaths?

Diagnostic Criteria for SLE

• Skin– Malar Rash– Discoid Rash– Photosensitivity– Oral/Nasal Ulcers

• MSK– Non-erosive arthritis

• Serologies– ANA– Anti dsDNA, anti-smith,

APLA

• Cardiopulmonary– Serositis

• Renal– Proteinuria or cellular casts

• CNS– Seizures, psychosis, etc

• Heme– Hemolytic anemia OR– Leukopenia OR– Lymphopenia OR– thrombocytopenia

But ALSO: constitutional complaints, abd pain, alopecia, vasculitis, raynaud’s, eye problems, etc.

1

76

89

10

11

23

4

5

Autoantibodies• Most specific for SLE

– Anti Smith Ab• Prognositic for SLE and kidney disease

– Anti ds DNA Ab• APLA – bleeding or clotting?

– Clotting, veins AND arteries• ANCA?

– Wegener’s granulomatosis, Microscopic polyangiitis, Churg-Strauss syndrome• Wegener’s: c-ANCA, anti-PR3• Microscopic Polyangiitis: p-ANCA, anti-MPO

• Hematuria and Hemopytisis, not ANCA related– Goodpasture’s, anti-GBM Ab disease– Could also be SLE

• Taking hydralazine, now have arthritis and malar rash?– Anti-Histone Ab for drug induced Lupus

• Anti-Mitochondrial Ab– Primary Biliary Cirrhosis

• Anti-Endomysial Ab and Tissue Transglutaminase Ab– Celiac disease

• Autoimmune Hepatitis– Anti Smooth Muscle Ab

Wegener’s Granulomatosis

Autoantibodies + Pearls• Limted Scleroderma – Ab and Symptoms?

– Anti-Centromere Ab– CREST [calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasias]

• Diffuse Scleroderma -- Ab– Anti SCL-70

• Autoimmune cause of oral and genital ulcers?– Behcet’s Syndrome

• Young Asian female with loss of radial pulses, constitutional symptoms?– Takayasu’s Arteritis

• 85F with amaurosis fugax, headaches, scalp tenderness on same side, Dx? Tx? Work up?– Temporal Arteritis AKA Giant Cell Arteritis– ESR very high– Treat with high dose steroids – IMMEDIATELY; to prevent blindness– Get a temporal artery biopsy

• I have IBD and now an elevated bilirubin and alkaline phosphatase? – Primary sclerosing cholangitis

Takayasu’s Arteritis

I had a URI, now I have . . .

I got a URI, now I have a rash and bloody urine . .

• Henonch Shonlein purpurua• IgA Nephropathy [synpharyngitic]

• Post Streptococcal GN occurs after the pharyngitis

• 29 AA Fw/ 2mo of arthralgias of knees, elbows, hands, and swelling in legs. BP 150/95. HR 79. 2+ pitting LEE.

• HCT 35%; C3 60; C4 12; ANA positive; 24 Urine protein 4.6g. Urine sediment with erythrocyte casts, oval fat bodies.

• DDx? Likely Dx?• Work-up?

Nephrotic Syndrome• >3.5g of protein in 24h U collection• Can present with either nephrosis or nephritis• Causes of this Syndrome

– Diabetic Nephropathy– Minimal Change Disease – think young, Kids!; heme CA– Membranous Nephropathy – HBV, solid tumors, class V SLE nephritis,

NSAIDS– FSGS [obesity, HIV, idiopathic, heroin]– Myeloma– Amyloidosis

• Urine Sediment: oval fat bodies or benign• General Tx: ACEI, diurese, treat underlying illness

Oval Fat Bodies

• 66F with severe pain in L calf, sudden onset. Has RA of many joints. Has had many knee injections because of pain and effusions with triamcinolone. Now is treated with etanercept and methtotrexate. PE with large R knee effusion and L knee is smaller in size. The knee was similar in size to the R until the pain began. The L calf is 5cm larger in diameter than the R.

• Diagnosis?

RA

• Chronic, symmetric, inflammatory, destructive• Joints – PIPs, MCPs, wrists, knees, ankles, MTPs• C1-C2 instability – A Classic Question• S/Sx: – Constitutional: fever, weight loss, malaise– Pulm: ILD, nodules, fibrosis, pleuritis +/- effusions– Vascular: leukocytoclastic vasculitis– Cardiac: pericarditis, myocarditis

Seldom Seen

Nodules

Diagnostic Criteria for RA? 4 out of 7

• AM Stiffness >1h• Hand Joint Arthritis >6wk• Rheumatoid Nodules• X-ray changes – erosions or periarticular

osteopenia• Arthritis of >3 joints simultaneously >6wk• Symmetric involvement >6wk• +RF [but check the CCP]

Diagnosis?

Diagnosis?

Gout: Negatively Birefringent Needle Shaped Crystals

Pseudogout = Calcium Pyrophosphate Deposition DiseaseWeakly Positive Birefringent Rhomboid Shaped Crystals

What Is This?

Gout• SHELF: obese, drinking, male, middle aged, carnivorous• Acute Monoarticular Arthritis

– 1st MTP = Podagra– Overlying skin, dusky, red, tense, red– Also at feet, ankles, knees

• Don’t check serum uric acid during a flair!• The joint fluid: lots of WBCs [20-100k]; majority are PMNs. Find

the crystals! Get a Gram Stain!• Tx

– Acute: NSAIDS, colchicine, maybe steroids– Chronic: decrease purine intake, daily colchicine

• Allopurinol or probenecid• not until acute issues resolved; tx w/ colchicine or nsaids concominantly while

reducing UA levels

Calcification of cartilage as seen on X-ray?

Chondrocalcinosis of CPPD or Pseudogout

26F w/ multiple sexual partners

• Migratory polyathralgias• True inflammation tenosynovitis• Synovial fluid 50K WBC, mainly PMNs• Blood Cultures growing GN diploocci

Cause?Treatment?

Disseminated Gonococcal Infections• Most common infectious arthritis of sexually

active young adults• Preceded by mucosal infection – can be ASx– Cervicitis– Urethritis– Pharyngitis

• Migratory Polyarthralgias• Tx with ceftriaxone x7d, must also treat for

Chlamydia – azithromycin or doxycycline

“Doc, since I was 20 I’ve had low back pain, especially in the morning . . .”

SI JOINT

Picture 1

And his spine films . . .

PICTURE 2

What does Seronegative Spondyloarthropathy Mean?

• Absence of rheumatoid factor, autoantibodies• Inflammatory! Aseptic. ESR elevated• Has a tendency to affect spine, SI joint, but also other joints• Also can affect eyes [uveitis, scleritis, iritis, conjuntivitis]• Associated with HLA-B27• Think of 4 illnesses

1. Ankylosing spondylitis2. Psoriatic arthritis3. Enteropathic artritis4. Reactive arthritis

Ankylosing Spondylitis

• Classically: starts in late teens, early 20s; gradual onset low back pain, worse in AM [inflammatory!], improves with movement/exercise

• Progressive involvement of spine, starting at SI Joint [picture 1] erosions and sclerosis

• Also inflammation at insertion sites for tendons/ligaments enthesitis– Achillies pain– Plantar Fasciitis– Spine Bamboo Spine [picture 2] – spinal ligament calcification

and bridging syndesmophytes• Also could see uveitis

PIP pains and scaly papules on forearm?

Psoriatic Arthritis

• Can have various presentations . . . – Monoarticular/dactylitis – Esp DIP– Polyarthritis– Axial involvement – like AS

• Arthritis can preceded skin findings by years• Enthesitis• Pitting fingernails• Joint Films– ‘Pencil in Cup’ deformity at DIPs

And the 2 Other Seronegative Spondyloarthropathies

Reactive Arthritis• Follows GU or GI infection• The Triad

– Seronegative arthritis– Urethritis– Conjunctivitis

• Males > Females

Enteropathic IBD Associated• Can look just like AS• Also can see

– Erythema nodusum– Pyoderma gangrenosum

Erythema Nodosum

Pyoderma Gangrenosum