A. Remitting Seronegative Symmetric Synovitis With Pitting Edema (RS3PE) [1]

1. RS3PE Syndrome
   1. M:F 2-4:1, Mean age ~70 years
   2. Patients are usually caucasian
   3. ~70% HLA-B7 positive (Relative risk ~4.4X)
2. Symptoms
   1. Acute onset
   2. Symmetric involvement
   3. Wrists, carpal joints, flexor tendon sheaths, small hand joints
   4. Elbows, shoulders, hips, knees, ankles may be involved
   5. Pitting edema on dorsum of hand
3. Laboratory
   1. Persistent RF negative
   2. Mild anemia with decreased serum albumin
   3. Elevated ESR
4. Treatment
   1. NSAIDs ineffective
   2. Edema resolves with low doses glucocorticoid, such as 10mg prednisone qd
   3. Hydroxychloroquine (Plaquinil®, 200-400mg/d) effective
   4. High dose salicylates may also be effective
   5. Disease may be self limted
   6. Asymptomatic flexion contractures of fingers and wrists often occur

B. Palindromic Rheumatism [2]

1. Characteristics
   1. Patients usually older (>50 years) develop acute transient migratory oligoarthritis
   2. Extremely painful, very red joints with periarticular erythema as well
   3. Attacks typically afebrile, lasting hours to days
   4. MCP/PIP > Wrists > Shoulders > Knees > Ankles > Feet
2. Unlike rheumatoid arthritis (RA), no female predominance (M ~ F)
3. ESR often increased; usually inflammatory synovitis
4. Treatment
   1. Excellent response initially to low dose prednisone (10-15mg/d)
   2. Response to NSAIDs variable
   3. Often controlled with hydroxychloroquine 400mg/d orally
   4. Second line therapy required in many patients after 2 years [3]
5. Prognosis
   1. >50% of patients eventually develop true Rheumatoid Arthritis
   2. ~20% of patients spontaneously remit
   3. Remainder of patients at higher risk for development of another rheumatologic disease
   4. Many will develop systemic lupus, Sjogren's Syndrome, or other autoimmune disease

C. Elderly Onset Rheumatoid Arthritis (EORA)

1. Characteristics
   1. Usually affects older patients (>55 years old)
   2. Unlike classical RA, EORA affects larger joints including elbows and shoulders
   3. Patients may have swollen, tender joints
   4. Myalgia symptoms may be prominant; polymyalgia rheumatica should be considered
2. Laboratory
   1. ESR is usually normal or slightly elevated
   2. Rheumatoid factor (RF) is positive in ~50% of cases
   3. Radiographs may show joint erosions
3. Treatment
   1. NSAIDs may be effective but should be used very cautiously in older persons
   2. Low dose glucocorticoids are often very effective and can be tapered
   3. Hydroxychloroquine (Plaquenil®) may be used as an adjunctive therapy
   4. Hydroxychloroquine is safe, but requires 3-6 months for activity
   5. Other second line agents should be used very cautiously in this group

D. The SAPHO Syndrome [4]

1. Components
   1. Synovitis
   2. Acne
   3. Pustulosis
   4. Hyperostosis
   5. Osteitis
2. Skin lesions may be delayed
3. Unclear etiology of syndrome

References

1. Russell EB, Hunter JB, Pearson L, McCarty DJ. 1990. J. Rheumatol. 17(5):633
2. Guerne PA, Weisman MH. 1992. Am J Med. 93(4)451
3. van Schaardenburg D, Valkema R, Dijkmans BA, et al. 1995. Arthritis Rheum. 38(3):334
4. Kahn MF. 1995. J Rheumatol. 22:2017