For Uveitis, What JIA is it usually associated with? What are the general symptoms? What can predict a higher risk? What three things can be caused by uveitis?

Uveitis Oligoarticular JIA Often asymptomatic ANA positivity predicts higher risk Can lose sight Presentation Posterior synechiae: irregular border of pupil Hypopyon: collection of inflammatory fluid at bottom of iris Band keratopathy: cloudy white band across iris

Rheumatology 2 Flashcards by Rahul Gupta

What are possible differentials of monoarticular/oligoarticular (less than 4 joints) arthritis with fever and without fever?

Monoarticular/Oligoarticular Arthritis (≤ 4 joints)
- Fever: infection and systemic onset JIA
- No Fever: trauma, JIA, Lyme arthritis, Psoriatic arthritis

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What are possible differentials of polyarticular (5+ joints) arthritis with fever and without fever?

Polyarticular Arthritis (≥ 5 joints)
- Fever: infection, SLE, systemic onset JIA
- No Fever: JIA or other rheumatic disease

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What is the umbrella term for most joint pain in juveniles?

Juvenile Spondylarthropathies: umbrella term for joint pain in juveniles

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For JIA (Juvenile Idiopathic Arthritis), what is the criteria (age and duration), genetics, and pathophysiology?

JIA (Juvenile Idiopathic Arthritis)
- Criteria: age at onset is < 16 yo, duration is > 6 weeks, and all other causes are ruled out
- Genetics: HLA types + environmental factors
- Pathophysiology is very similar to RA: proliferation of synovium and increase in vascularity

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What are some characteristics of oligoarticular JIA?

Oligoarticular JIA: asymmetric joints, muscles atrophy, and limb-length discrepancy, often ANA positive (also found in SLE)

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What are some characteristics of Polyarticular RF JIA?

Polyarticular RF JIA
- Can be RF negative or positive

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Is RF+ or RF- polyarticular JIA more damaging?

RF positive can be more damaging because it follows the course of adult RA

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What are the symptoms associated with systemic JIA?

What are the lab tests of systemic JIA?

What is the fever pattern associated with systemic JIA?

Systemic JIA: associated with rash, fever lasting > 2 weeks, and microcytic anemia
- Elevated WBCs, D dimer, AST/ALT, LDH (tissue damage factors)
- Quotidian fever pattern: spikes at night

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What are the symtoms/features associated with psoriatic arthritis?

Psoriatic Arthritis: dactylitis (big swollen digits), nail abnormalities, and/or family history of psoriasis

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What are the charactersitics of enthesitis related JIA?

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List 3 extra-articular complications of JIA.

Uveitis
Growth abnormalities
Macrophage Activation Syndrome

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For Uveitis,

What JIA is it usually associated with?
What are the general symptoms?
What can predict a higher risk?
What three things can be caused by uveitis?

Uveitis
- Oligoarticular JIA
- Often asymptomatic
- ANA positivity predicts higher risk
- Can lose sight
- Presentation
  - Posterior synechiae: irregular border of pupil
  - Hypopyon: collection of inflammatory fluid at bottom of iris
  - Band keratopathy: cloudy white band across iris

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For growth abnormalties in JIA, what are the two types of growth abnormalities and what JIA are they associated with?

Growth abnormalities
- Oligoarticular JIA: limb-length discrepancy
- Polyarticular JIA: micrognathia – damage to the mandibular growth plate

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For Macrophage Activation Syndrome (MAS), what JIA is it associated with? What is the hallmark appearance on a slide?

Macrophage Activation Syndrome
- Systemic JIA
- Hallmark: macrophages ingesting other hematopoietic cells on bone marrow biopsy

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What is the general approach to any JIA (3 treatments)?

NSAIDs
Corticosteroids (prednisone)
DMARDS
- Methotrexate
- Hydroxychloroquine

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What are the differential diagnoses/clinical features of acute arthritis (5 of them)?

Differential Diagnoses
- Infection: single event with inflammation and fever
  - Accompanied with systemic illness
- Trauma: possible hemarthrosis (blood in joint)
- Crystals: may be accompanied with previous episodes and fever
- Inflammatory arthritis: usually polyarticular, but has to start somewhere
- Malignancy

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What are the two types of crystals found in synovial fluid?

Monosodium urate (MSU)
- Negative birefringent
- Pointed ends of crystals
Calcium pyrophosphate dehydrate (CPPD)
- Positive birefringent
- Blunt ends of crystals

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For synovial fluid analysis, provide the color, viscosity, WBC count, neutrophils, and glucose level for:

Normal
Non-inflammatory
Inflammatory
Septic
Hemmorrhagic

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What are the risk factors of gout? There are 4 main ones and know how they generally work.

Risk factors
- Ethanol: increased nucleotide catabolism and decreased
- Diuretics: thiazides enhance tubular reabsorption of uric acid
- Salicylates: aspirin at low doses blocks excretion of urate
- Lead: decreases renal excretion

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What is the general pathogenesis of gout including the general immune response?

Increased production or decreased excretion → excess uric acid build-up (product of purine metabolism) → precipitation of MSU crystals into joint from → triggers immune response with IL-1, IL-6, and TNF-alpha

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Why is gout considered self-limiting?

Gout is self-limiting because it is coated with apoprotein B, which decreases inflammatory triggers

What occurs in the tophaceous phase of gout? What are tophi and what can they do to bones? How do they appear in x-rays?

Tophaceous Phase: collections of uric acid in soft tissues (ears, fingers/hands, feet, elbow)
- Can result in destructive, erosive changes to the joint and bone as tophi eat through
  - Hole punch look to bone
  - “Over-hanging edge”
- Looks a lot like rheumatoid arthritis, but differentiated by culture

Compare gout to rheumatoid arthritis (4 main differences)

Gout versus RA

RA has symmetric polyarticular and insidious pain
RA nodules instead of tophi
RA has prolonged morning stiffness
RA has +RF antibodiesNext

Compare gout to psedogout. 4 main differences

Pseudogout has CPPD crystals that are tubular/rhomboid shaped
Pseudogout has normal serum uric acid levels
Pseudogout has chondrocalcinosis on XR (Ca++ buildup on articular cartilage giving rise to “fuzzy” look) - SHOWN IN PIC ATTACHED
Pseudogout is treated with the same as acute gout

How can you modify the lifestyle of crystal arthritis?

* Risk factor modification: no alcohol, discontinue low dose aspirin

What are the acute treatments for crystal arthritis (gout and pseudogout)?

* Acute * NSAIDS * Steroids * Colchicine: mitotic poison that inhibits microtubules of neutrophils * ACTH injections (gout only)

What are the chronic treatments for crystal arthritis (gout and pseudogout)?

* Chronic * Uricosuric agents: increase solubility/excretion of uric acid * Probenecid * Uricase * Lesinurad * Allopurinol: xanthine oxidase inhibitor * Febuxostat: xanthine oxidase inhibitor

What is ankylosing spondylitis?

* Chronic inflammatory disease of the axial skeleton manifested by back pain and progressive stiffness of the spine * Can affect peripheral joints

List members of the HLA-B27-associated family of diseases (3 of them)

* Psoriatic arthritis, IBD-related arthritis, and reactive arthritis

What are the musculoskeletal symptoms of ankylosing spondylitis? 6 total.

* Inflammatory low back pain * Improvement with exercise and gets worse with rest * Hip, groin, and buttock pain (hip pain is associated with worse prognosis) * Enthesitis are inflamed enthuses (areas where tendons and ligaments attach to bones) * Peripheral arthritis * Dactylitis

What are the two main tests that we use to diagnose back deformities (ankylosing spondylitis)?

* Schober Test: forward flexion of lumbar spine * The difference in length of the lumbar spine between the standing and flexed position – must be greater than 4cm * Occiput to wall: distance of occipital bone to wall when standing against a wall

List 3 extra articular manifestations of ankylosing spondylitis.

* Uveitis * Psoriasis * Cardiovascular disease

What is the non-pharmacological (3), pharmacological (3~), and other treatments for ankylosing spondylitis(1)?

* Non-pharmacological: smoking cessation, exercise, PT * Pharmacological: NSAIDs, TNF-alpha inhibitors * If peripheral arthritis, use sulfasalazine (DMARD) * Surgery

What are the three different types of manifestations including malignancy risk for **Dermatomyositis**?

* Cutaneous * Muscle * Systemic

What are the 3 cutaneous manifestations of dermatomyositis and what are their features??

* Periorbital violaceous or heliotrope rash with swelling: rash and swelling around eye * Gottron’s Papules: raised scaly and dry skin on extensor surfaces * Compared to psoriasis, this has rash on joints of fingers * Periungual erythema: abnormal capillaries at base of fingernail

What does Periorbital violaceous or heliotrope rash with swelling look like?

What does Gottron’s Papules look like?

What does Periungual erythema look like?

What is the pathology of dermatomyositis?

* Pathology: fatty replacement of muscle tissue, complement deposition, and inflammatory blue muscle cells present

What imaging is used for dermatomyositis?

* Imaging: MRI shows inflamed muscle tissue (more water = whiter muscles)

What lab tests are used for dermatomyositis? What antibodies do we look for?

* Laboratory tests: creatine kinase (CK), aldolase, and transaminase (AST and ALT but may be mistaken for liver diseases) * Anti-Jo1 and Anti-MDA5 – highly specific for certain subsets of myositis * Electromyography (EMG) – certain patterns characteristic of myositis

Distinguish features of juvenile versus adult dermatomyositis.

* Better prognosis and no cancer * Present with vasculitis and soft-tissue calcification (indicators of long-term complications)

For dematomyositis, what are the treatments for muscle and skin inovolvement (3 overall)?

* Glucocorticoids * Sunblock * Immunosuppressants: methotrexate, azathioprine, and B-cell targeting drugs (rituximab)

How rare and severe is scleroderma when compared to lupus?

* Scleroderma is rarer than lupus but higher mortality rate

What are the physical findings of scleroderma?

* **Sclerodactyly**: immobile fingers due to collagen accumulation in subcutaneous tissue * **Raynaud’s phenomenon**: reversible perfusion of fingers shown in a change of color (SEE PIC ATTACHED) * **Gangrene**: necrosis of fingers * **Periungual erythema**: abnormal capillaries at base of fingernail * **Esophageal dysmotility**: aspiration due to weakness in peristalsis muscles of esophagus * Fluid can’t go down * I**nterstitial lung disease**: inflammation of lungs followed by fibrosis * **Kidney issues**: vascular fibrosis with lack of lumen * Onion-skinning look

What three reasons can be argued for classifying scleroderma as an autoimmune disease?

* Why it might be an autoimmune disease * Associated with autoantibodies – ANA positivity * Shares susceptibility genes with lupus * Treated with immunosuppressive drugs

What three reasons can be argued for NOT classifying scleroderma as an autoimmune disease?

* Why it might not be an autoimmune disease * It may be made worse by high dose prednisone * Tissues show more fibrosis than inflammation * Many abnormalities are vascular

What are the major clinical manifestations of Sjorgen's Syndrome (SS)?

* Sicca complex: dry eyes and dry mouth * Can get oral infections: candidiasis

What are the diagnostic approaches (lab tests and pathology) to investigate for Sjorgren's?

* Autoantibodies: SSA (Ro) and SSB (La) (SS = Sjorgren’s Syndrome) * Lip biopsy: salivary glands will be infiltrated with inflammatory cells

What are the non-pharmacological for Sjorgren's (for dry mouth and eyes)?

* Non-pharmacological treatments * Dry-mouth: dental care and water * Dry-eyes: artificial tears and occlusion of the lacrimal puncta (blockage of tear duct)

What are the pharmacological for Sjorgren's (for dry mouth and eyes)?

* Pharmacologic treatments * Dry-mouth: cholinergic agents to stimulate salivation * Dry-eyes: topical cyclosporine