7. Juvenile Idiopathic Arthritis / Rheumatoid Arthritis

MC rheumatic disease under 16 years

Juvenile idiopathic arthritis

Juvenile idiopathic arthritis (define)

Inflammatory arthritis age <16 years that lasts >6 weeks

Presentations of juvenile idiopathic arthritis (3)

1. Pauciarticular — MC

2. Still's Disease

3. Polyarticular

S/S & lab findings of Still's Disease (sJIA)

- Evanescent (very short time)

- Lymphadenopathy, pleural effusions

- Intermittent fevers

- Salmon colored rash

- Marked leukocytosis

S/S & lab findings of pauciarticular JRA

- Girls <4 years

- Monoarticular OR oligoarticular arthritis

- Uveitis (anterior) — Asymptomatic

- ANA positive

ANA positive with workup of JRA...

Refer to ophthalmology for uveal tract inflammation

S/S & lab findings of polyarticular JRA

- Resembles classic adult RA: Chronic, disabling

- Arthritis of ≥5 joints — Usually symmetric

Long term complication of Still's Disease (sJIA)

Micrognathia — Receding chin

Management of Still's Disease (sJIA)

- PT / exercise

- Naproxen & ibuprofen

- DMARDs

- Corticosteroids (beware of daily steroids & growth)

MC inflammatory arthritis with symmetric involvement of small joints of hands & feet

Rheumatoid arthritis

What joints are MC affected with rheumatoid arthritis? (& more)

- Peripheral hands & feet — MCPs, PIPs, MTPs

- Ankle, subtalar

- C1-C2 (late stage)

- TMJ

What lab finding is associated with extraarticular manifestations of rheumatoid arthritis?

Seropositive RA

Seronegative RA

Difficult to diagnose w/o erosions → Always consider calcium pyrophosphate deposition (CPPD)

Goal of management of rheumatoid arthritis

Early therapy → Remission / low disease activity

Risk factors for rheumatoid arthritis

- Smoking

- Periodontal disease

- Genetics — HLA

- Female, post-menopause, anti-estrogens

- Respiratory exposures — Pollution, silica dust, COPD, asthma

- Intestinal health — Chronic diarrhea

How to distinguish RA & OA on PE?

RA — Joints soft to palpation (vs. hard with OA)

Demographic associated with rheumatoid arthritis

Women of childbearing age

S/S & PE of rheumatoid arthritis

- AM stiffness >30 mins

- Improves with activity

- Ulnar drift at MCPs

- Periodontal disease

- MCP, PIP (MC index & middle) & MTP joint involvement — Soft to palpation

Extra-articular conditions associated with rheumatoid arthritis

- Lung / skin rheumatic nodules

- Anemia of chronic disease

- Ocular — Dry eye

Domains of diagnosis of rheumatoid arthritis

1. Joint involvement — Peripheral joints of hands & feet

2. Serology: RF & anti-CCP

3. Acute phase reactants: ESR & CRP

4. S/S duration: >/< 6 weeks

**Combination of clinical, joints, location, labs, S/S duration

Hallmark S/S of rheumatoid arthritis (& define)

Synovitis — AI inflammation of synovial membrane

Diagnostic criteria for rheumatoid arthritis

- >1 large OR small joint of hands / feet OR positive squeeze test (tenderness gently squeezing across MCP / MTPs)

- >6 weeks

**Both → Refer to rheum

Joint involvement emergency with rheumatoid arthritis

Cricoarytenoid

Common comorbid conditions with rheumatoid arthritis

- CVD

- HTN

- Osteoporosis

- Depression

Protective factors of rheumatoid arthritis

- Parity, breastfeeding

- Mediterranean diet, tea, very small alcohol consumption

Prognosis of rheumatoid arthritis

- Gradual progression

- AM stiffness / pain d/t fluid buildup during inactivity

- Joint pain & deformities (bone / cartilage damage) → Gradual loss of function

Diagnostic workup of joint pains

- Rheumatoid factor (RF)

- Anti-CCP

- ANA

- Lyme titer

- ParvovirusB-18 antibody, IgG, IgM

- ESR / CRP

**Positive RF or CCP does NOT always indicate RA

What lab is indicative of a worse prognosis of rheumatoid arthritis?

High RF & anti-CCP

Anticyclic citrullinated peptide antibodies (Anti-CCP)

- Autoantibodies against CCPs: Fibrinogen, enolase, collagen II, vimentin

- Similar OR higher sensitivity for RA vs. RF (can be seen earlier)

-

**NOT all RA are anti-CCP (& RF)

What is recommended for the management of ALL types of arthritis?

Exercise — Strong muscles → Improved articulation of joints

Diet / supplements & rheumatoid arthritis

- NO foods linked to RA BUT weight loss / nutritional counseling recommended

- Fish oil can be associated w/ lower risk of RA

**Screen for Vit D & B12 deficiencies

Initial management of S/S of RA awaiting rheumatology consult

- NSAIDs — Pref for COX2

- Acetaminophen ≤4 g/d

- Short term systemic corticosteroids (low-dose, shortest time possible)

Benefits of steroid use for rheumatoid arthritis

Bridge for DMARDs

DMARDs AKA

Disease-Modifying Antirheumatic Drugs

Benefits of DMARDs

- Decrease RA severity, disability, & mortality

- Control of inflammation may decrease cardiac & malignancy risks

Anchor drug for treatment of rheumatoid arthritis

Methotrexate

Pharmaceutical management of rheumatoid arthritis

- Methotrexate, hydroxychloroquine, leflunomide

- TNF blockade

- T-cell & B-cell blockades / drugs

- IL1 & IL6 blockers

- JAK inhibitor

ADRs of methotrexate

Hepatotoxicity, teratogenicity

**Monitor CBC, liver transaminases, serum Cr

ADRs of hydroxychloroquine

Retinal damage, cardiac dysarrhthmias, prolonged QT, Toursades

ADRs of IL6 receptor blockers

Elected lipids, GI perforation

ADRs of JAK inhibitors

Shinges. GI perforation, lipid abnormalities

Immunizations with rheumatoid arthritis

- Reduced responses & meds can blunt responses further

- Vaccines can precipitate flare

- Influenza & pneumococcal vaccinations recommended

**AVOID live virus with use of immunomodulators

What vaccines should be avoided with those on immunomodulating agents?

Live vaccines (intranasal flu, MMR, yellow fever, typhoid, oral polio)

**Wait at least 2 weeks after vaccines before initiating immunomodulators

What vaccines should be avoided with those on biologic agents?

Zoster — May be given to patients on MTX and prednisone <20 mg/d

Red flags with rheumatoid arthritis

- Fever & infection with use of steroids, methotrexate (MTX), leflunomide, biologics, small molecule DMARDs

- Isolated monoarthritis OR inflammation of total joint replacement → Septic arthritis

- Dyspnea & cough with MTX (MTX pneumonitis)