FELTY SYNDROME- 2-4% RA patients develop

Characterized by:

1. RA (RF+)(nodules, vasculitis)

2. splenomegaly

3. granulocytopenia

Pathophysiology

- splenic sequestration and subsequent granulocyte destruction

- granulocyte surface antigen autoantibodies

- autoantibodies against granulocyte growth factor G-CSF

RA – vasculitis

Felty syndrome – vasculitis

Systemic JRA (Still’s d.)

15 month female presented with rash and chronic recurring fever for 3 weeks.

PSORIATIC ARTHRITIS

● Autoimmune disease

►An inflammatory arthritis associated with psoriasis, usually RF-neg.

●slightly over 1 million in US. (about 10% of those that have psoriasis)

●men and women affected equally

●mostly develops in persons aged 35-55 years

●more common in white persons than other races

● characterized by flares and remissions.

●psoriasis usually preceeds arthritis ---sometimes by up to 20 years ---in 15%...arthritis appears before psoriasis ●80% with PA have nail changes - pitting - lines - yellow spots - onycholysis (loosening of nail)●psoriasis – mild (few spots) to severe (all over body)●30% develop eye involvement (inflammation)

PSORIATIC ARTHRITIS (PA)

PSORIATIC ARTHRITIS

►Psoriatic arthritis affects the ligaments, tendons, fascia, and joints. The most common patterns are:

■ assymetric oligoarticular type○Usually, the digits of the hands and feet are affected first, with inflammation of the flexor tendon and synovium occurring simultaneously, leading to the typical "sausage" appearance (dactylitis). ○Usually, fewer than 5 joints are affected at any one time.

Comparison between psoriatic arthritis and rheumatoid arthritis in both hands and feet.

■ Symmetrical polyarthritis type  ○ rheumatoidlike pattern; most common type (45%). The hands, wrists, ankles, and feet may be involved. ○ It is differentiated from rheumatoid arthritis (RA) by

- distal interphalangeal (DIP) joint involvement, - the absence of subcutaneous nodules, - negative test result for rheumatoid factor (RF).

This condition generally is milder than RA, with less deformity.

■ Spondylitis (with or without sacroiliitis) ○ about 8-10% of patients with psoriatic arthritis○ male predominance○ vertebrae are affected asymmetrically (involvement differs from that observed in ankylosing spondylitis.)

PSORIATIC ARTHRITIS

■ Juvenile psoriatic arthritis   - accounts for 10-20% of childhood arthritis and is monoarticular at onset. - mean age of onset is 9-10 years, with a female predominance. - The disease is usually mild, although occasionally it may be severe and destructive, progressing into adulthood. - In 50% of children, the arthritis is monoarticular; DIP joint involvement occurs at a similar rate.