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Rheumatology

With Kartik Rangaraj MD

Nailah Rahmah, nailahrahmah1997@gmail.com


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Overview

Nailah Rahmah, nailahrahmah1997@gmail.com


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Rheumatic Diseases

Historically, the diseases within the field of Rheumatologists also treat:


rheumatology have been called collagen- • Osteoarthritis (OA)
vascular diseases (a now antiquated term).
• Crystalline arthropathies (gout/pseudogout)
Within this, several subsets exist: • Septic arthritis
• Connective Tissue Diseases
(CTDs; i. e., lupus)

• Seronegative Spondylarthropathies CTDs Vasculitis SN SpA


(SN SpA; i. e., ankylosing spondylitis)

• Vasculitides (i
Most CTDs can cause vasculitis,
not vice versa

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Rheumatic Diseases

Rheumatologic diseases affect the extracellular components of tissues in almost every organ.
• Joints (arthralgias, arthritis)

• Skin (rash)

• Nerves/muscle (neuritis, myositis)

• Kidneys (nephritis)

• Lung, heart, gut, brain

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Lecture Outline
Non-Autoimmune Arthritis Vasculitis
• Osteoarthritis (OA) • Large vessel • ANCA-associated
• Crystalline arthropathies (gout/pseudogout) • Medium vessel • Non-ANCA-associated
• Septic arthritis • Small vessel

Seronegative Spondylarthropathies
• ankylosing spondylitis (AS) We will break each condition down into these sections:
• Reactive arthritis 1. Definition
• Psoriatic arthritis (PsA) 2. Pathogenesis
• Inflammatory bowel disease-associated arthritis 3. Signs & symptoms
4. Diagnosis
Connective Tissue Diseases
5. Treatment
• Rheumatoid arthritis (RA)
• Systemic Lupus Erythematosus (SLE)
• Scleroderma/ phenomena
• Syndrome (SS)
• Inflammatory myositis
(polymyositis/dermatomyositis)

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Rheumatic Diseases
Non-Autoimmune Arthritis

With Kartik Rangaraj MD

Nailah Rahmah, nailahrahmah1997@gmail.com


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Osteoarthritis

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Approach to Arthritis
Joint pain is a common symptom in rheumatic disease.
This is how rheumatologists approach this issue:

Arthritis vs. arthralgias


• Arthritis joint-centered (articular) pain

• Arthralgias sensation of joint pain but pain around joint (periarticular)

Acute vs. chronic (  6 weeks)


• Symmetrical vs. asymmetric
Forms basis of how to
• Monoarticular vs. polyarticular describe arthritis
• Inflammatory vs. noninflammatory

AM stiffness > 1 2 hrs: key feature of systemic inflammatory (read: autoimmune) arthritis!

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Osteoarthritis

Osteoarthritis is a chronic, asymmetric, noninflammatory,


degenerative arthritis (wear-and-tear arthritis) associated with
articular cartilage damage and formation of new bone at joint
surfaces and margins.

Definition It commonly affects joints that are subject to repetitive use and
weight-bearing joints:
• Small joints of the fingers (CMC of thumb, DIP, PIP)
• Knees
• Hips
• Spine

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Osteoarthritis

• Very complex orchestration of biomechanical, biochemical,


and cytokine-mediated changes

• Obesity, which increases the work-load on weight-bearing joints


and increases the risk for OA

• Incidences of OA increases with age


Pathogenesis

• In contrast to RA, pain in OA worsens with use

• Classically, DIP and PIP joints (


respectively) are involved; clinically, CMC of thumb is usually first
hand joint involved

• OA frequently involves the knees, spine, and hips


Signs & symptoms • Crepitus of affected joints

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Osteoarthritis

Marshall M, Jonsson H, Helgadottir GP, Reliability of Assessing Hand Osteoarthritis on Digital Photographs /
detailedresult.php?img=PMC4153954_acr0066-0828-f1&query=osteoarthritis+of+the+hand&it=xg&lic=by&req=4&npos=1, cropped, CC BY 3.0
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Osteoarthritis

Shah N, Mini-subvastus approach for total knee arthroplasty in obese patients., https://openi.nlm.nih.gov/detailedresult.php?img=PMC2911930_IJOrtho-
44-292-g001&query=osteoarthritis+of+the+knee&it=xg&lic=by&req=4&npos=32, cropped, CC BY 2.0
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Osteoarthritis

• ESR is normal.

• Rheumatoid factor is not present.

• X-rays reveal subchondral sclerosis and osteophyte formation


(bone spurs).

• Osteophytes of the DIPs: nodes


Definition

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Osteoarthritis

• Acetaminophen (Tylenol) and NSAIDs are the mainstay of


therapy.

• Weight loss, physical therapy, and supports

• Orthopedic surgery for joint replacement

• No role for PO steroids

• Can inject intraarticular steroids (very limited relief) or


Treatment hyaluronic acid

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Gout & Calcium Pyrophosphate
Deposition Disease (CPPD)

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Gout

Gout is an acute, inflammatory, monoarticular, asymmetric arthritis


due to deposition of monosodium urate crystals intraarticularly.

Definition

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Gout

Phagocytosis of urate crystals by neutrophils causes release of


inflammatory mediators via activation of the inflammasome
worry, the inflammasome will not be on your boards).

Hyperuricemia (uric acid > 7 mg/dl) is a risk factor for the development
of gout.

Pathogenesis Disorders of uric acid metabolism involve one of the following:


• Overproduction or under-excretion
• Under-excretion much more common (90 % of gout pts)

Associated with chronic kidney disease (CKDs), HTN, DM2,


dyslipidemias, obesity, and transplant pts (especially cardiac, related
to cyclosporine).

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Gout

Cardinal symptom: sudden onset of pain


• Pain is very severe
stand the weight of a bed sheet on the affected joint)

• The joint is inflamed (tender, red, hot, & swollen)

• Most common joint affected: 1st MTP joint


• This is so characteristic of gout that it has
Signs & symptoms its own name: podagra

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Gout

Tophi (subdermal urate crystal


deposition) may be found.
• Particularly along the pinna of ear,
elbows

Recurrent episodes of gout can lead


to a chronic arthritis.
• Each subsequent flare involves
Signs & symptoms more joints than prior flare

Roddy E, Revisiting the pathogenesis of podagra: why does gout target the foot?, https://openi.nlm.nih.gov/detailedresult.php?img=PMC3117776_1757-
1146-4-13-2&query=Gout&it=xg&lic=by&req=4&npos=4, resized, CC BY 2.0
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Gout Tophi

F.l.t.r.: Inani K, Mernissi F, [Gouty tophus]., https://openi.nlm.nih.gov/detailedresult.php?img=PMC4189869_PAMJ-17-250-g001&query=Gout+%26ndash%3B


+Tophi&it=xg&lic=by&req=4&npos=41, resized, CC BY 2.0; Michael McCullough, Gouty Tophi in the Helix of the Ear, https://flic.kr/p/9a2MrM, croped, CC BY 2.0
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Gout
• Analysis of the synovial fluid obtained by arthrocentesis

• Synovial fluid analysis reveal -


crystals
• MSU crystals are negatively birefringent under
polarized light.

Diagnosis
Although hyperuricemia is a risk factor for gout, uric acid levels are
neither diagnostic nor reliable during acute gouty attacks.
• Normal uric acid levels are seen during flares as most of it is in
immeasurable crystal form!

• Only 10 % of hyperuricemic pts ever develop gout

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Gout

Acute gouty attack

• NSAIDs (e.g. indomethacin)

• Colchicine for patients who cannot tolerate NSAIDs


• Colchicine causes diarrhea as one of its major side
effects; also remember myopathy and pancytopenia
as important side effects for exam purposes.

• Oral steroids (e.g., prednisone) for patients who cannot tolerate


Treatment NSAIDs or colchicine (advanced CKDs)

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Gout

Chronic gouty attacks

• Allopurinol and febuxostat can be used for patients with


hyperuricemia who are overproducers based on serum uric
acid level.
• Goal: serum uric acid < 6 mg/ml

• If urinary uric acid > 1000 mg/day, pts at risk for acute
obstructive uropathy

• Should not be started until several weeks after an acute gouty


attack.

Treatment • Need dose adjustment for chronic kidney disease.

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Gout

What if maxed out on allopurinol and still have high uric acid?

• Uricosuric drugs (furosemide, probenecid, sulfinpyrazone)


increase urinary uric acid excretion.

• Uricase treatment

• Anakinra (IL-1 antagonist)

Treatment

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Calcium Pyrophosphate Deposition Disease

Pseudogout is an acute, inflammatory, symmetric arthritis due to


deposition of calcium pyrophosphate crystals intraarticularly.

Definition

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Calcium Pyrophosphate Deposition Disease

• Deposition associated with high calcium and phosphate


concentrations
• Most commonly seen in large joints; mainly affects older patients
• See chondrocalcinosis in menisci of knees, pubic symphysis, and
wrists
• Associated with osteoarthritis and metabolic disorders that alter
calcium metabolism:
Pathogenesis • Hyperparathyroidism • Hypophosphatasia
• Hemochromatosis •
• Hypothyroidism
• Hypomagnesemia

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Calcium Pyrophosphate Deposition Disease

• Patients typically present with acute symmetric arthritis.

• These episodes are usually triggered by surgery


(secondary to volume shifts) or severe illness.

• Chronic disease mimics osteoarthritis (pseudo-OA) or


rheumatoid arthritis (pseudo-RA).
Signs & symptoms

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Calcium Pyrophosphate Deposition Disease

• Calcium pyrophosphate crystals appear


rhomboid-shaped and are positively
birefringent under polarized light.

• Soft tissue calcification


(chondrocalcinosis) can be seen
Diagnosis on X-rays.

Taokinesis ,CC0 1.0


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Calcium Pyrophosphate Deposition Disease

• Acute flares treated similarly to gout


• NSAIDs, steroids

• Treatment of underlying disorder is most important.

Treatment

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Septic Arthritis

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Septic Arthritis

Serious (read: medical emergency!) acute, inflammatory,


asymmetric, monoarticular arthritis caused by bacterial
infection of joint space.

Why is this a medical emergency?


It can take only hours for the infection to destroy the
cartilage within the joint to the point that the joint is permanently
damaged.
Definition

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Septic Arthritis
Usually there is a nidus of infection:

• In diabetics, skin infection secondary to neuropathic changes

• In drug users, direct inoculation of blood Look for


endocarditis!

• In young adults, look for Gonococcus!

• In sickle cell pts, look for Salmonella and Staph!


Pathogenesis
• In trauma pts, look for Staph epidermidis!

• Obviously, immunosuppressed pts have every reason to


develop septic arthritis.

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Septic Arthritis

High risk of seeding joints with bacteria:


• Pts with underlying joint disease (esp. rheumatoid arthritics, pts
with prosthetic joints)

The two main group of organisms causing septic arthritis are:


• Gonococcus (Neisseria gonorrhoeae)
• Nongonococcal
• Staphylococcal infection (most common S. aureus)

• Group A beta-hemolytic strep


Pathogenesis

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Septic Arthritis

• Acute, abrupt onset of monoarticular joint pain with redness,


swelling and warmth; associated with fevers/chills
• Mimics gouty arthritis

• Both septic and gouty arthritis can occur at the same time!
Signs & symptoms

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Septic Arthritis
Depending on bug, severity of symptoms vary:
• Nongonococcal (S. aureus) most aggressive in severity of pain
quickness of onset)
• Think gonococcal if arthritis is migratory and monoarticular in
nature
• Young sexually active patients with acute monoarthritis
considered to have GC arthritis until proven otherwise
• Slower onset of symptoms, usually over several days
Signs & symptoms • Patients may have symptoms of genital infection with
gonococcus
• If asymptomatic, can see periarthritis-dermatitis syndrome
• Patients may develop a pustular rash due to
disseminated GC infection
• Seen more in N. meningitidis
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Septic Arthritis

Gonococcal arthritis

• Positive sexual history

• Cultures of cervix/urethra/pharynx/rectum as appropriate


• Remember: gonococcus is hard to culture, so if
inappropriately performed by lab, these results
can be negative.

• Joint aspiration necessary.


Diagnosis

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Septic Arthritis

Non-gonococcal arthritis

• Aspiration of joint fluid will reveal large numbers of WBCs and


Gram stain & cultures will likely be positive; they also need to
be analyzed for crystals.
• If patient received antibiotics prior to aspiration, this can
create a false negative culture.

• Blood cultures
Diagnosis

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Septic Arthritis

Gonococcal arthritis

• IV ceftriaxone, followed by oral antibiotics

Non-gonococcal arthritis

• IV drugs with Gram (+) coverage (vancomycin)

• Non-GC arthritis require drainage:


Treatment • Needle aspiration vs. open surgical drainage (ortho consult)

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