Syndrome

Report
Joints, Rheumatology, and the
Shelf
Paul Johnson
prepared by Ryan Sanford
Chief Lecture
The Joints
44F 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 Pain
Duration
Chronic
Acute
1.
2.
3.
4.
5.
6.
Infection [septic arthritis]
Trauma/Blood
Crystals! Gout and CPPD
Reactive
Parvovirus B19
Early Chronic
Inflammation?
Activity helps,
stiff in AM [>1h]!
Activity doesn’t
help
No = OA
# Joints Involved
Mono
1.
2.
Indolent infection
Early oligo/poly
Oligo
1.
2.
3.
Spondyloarthropathy
Indolent infection
Early poly
Poly
1.
2.
3.
RA = symmetric
SLE = symmetric
Systemic Sclerosis = symmetric
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 1
Discoid Rash 2
Photosensitivity 3
Oral/Nasal Ulcers 4
• MSK
– Non-erosive arthritis 5
• Serologies
– ANA 6
– Anti dsDNA, anti-smith, 7
APLA
• Cardiopulmonary
– Serositis 8
• Renal 9
– Proteinuria or cellular casts
• CNS 10
– Seizures, psychosis, etc
• Heme 11
–
–
–
–
Hemolytic anemia OR
Leukopenia OR
Lymphopenia OR
thrombocytopenia
But ALSO: constitutional complaints, abd pain, alopecia, vasculitis, raynaud’s, eye problems, etc.
Autoantibodies
•
Most specific for SLE
–
•
Prognositic for SLE and kidney disease
–
•
Anti ds DNA Ab
APLA – bleeding or clotting?
–
•
Anti Smith Ab
Clotting, veins AND arteries
ANCA?
–
Wegener’s granulomatosis, Microscopic polyangiitis, Churg-Strauss syndrome
•
•
•
Hematuria and Hemopytisis, not ANCA related
–
–
•
Primary Biliary Cirrhosis
Anti-Endomysial Ab and Tissue Transglutaminase Ab
–
•
Anti-Histone Ab for drug induced Lupus
Anti-Mitochondrial Ab
–
•
Goodpasture’s, anti-GBM Ab disease
Could also be SLE
Taking hydralazine, now have arthritis and malar rash?
–
•
Wegener’s: c-ANCA, anti-PR3
Microscopic Polyangiitis: p-ANCA, anti-MPO
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 Disease
Weakly 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.
2.
3.
4.
Ankylosing spondylitis
Psoriatic arthritis
Enteropathic artritis
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

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