Table 6.1 ClassiÞcation of noninfectious diseases associated with scleritis

1.Associated with systemic immune-mediated diseases

1.1. Vasculitides

Primarily smalland medium-sized vessel vasculitic diseases

Connective tissue diseases in which vasculitis may occur

Adult rheumatoid arthritis Systemic lupus erythematosus Relapsing polychondritis Juvenile idiopathic arthritis SjšgrenÕs syndrome Dermatomyositis

Inßammatory conditions in which vasculitis may occur Arthritis and inßammatory bowel disease Psoriatic arthritis

Primarily vasculitic (and/or granulomatous) diseases

Polyarteritis nodosa Beh•etÕs disease CoganÕs syndrome

Allergic granulomatous angiitis (ChurgÐStrauss syndrome)

Granulomatosis with polyangiitis (Wegener) SchšnleinÐHenoch purpura

Primarily large-sized vessel vasculitic diseases

Inßammatory conditions in which vasculitis may occur

Ankylosing spondylitis Reactive arthritis

Primarily vasculitic diseases Giant-cell arteritis TakayasuÕs arteritis

1.2. Miscellaneous Thyroid disorders Sarcoidosis

Vogt–Koyanagi–Harada syndrome Sympathetic ophthalmia

Atopy

2. Associated with dermatologic diseases

2.1. Rosacea

3. Associated with metabolic diseases

3.1. Gout

4. Associated with foreign bodies

5. Associated with chemical injuries

6. Idiopathic

This chapter focuses on the systemic and ocular manifestations of the noninfectious diseases that may be associated with scleritis. Furthermore, it analyzes the presence of other ocular manifestations occurring before, during, or after the onset of scleritis that may be helpful in diagnosing the underlying systemic disease.

6.1Systemic Immune-Mediated Disease-Associated Scleritis: Vasculitides

6.1.1Adult Rheumatoid Arthritis

Rheumatoid arthritis is a chronic inßammatory systemic disease of unknown cause. Although the synovial membrane of the joints is the main target of damage, patients often have involvement of extraarticular tissues, such as eyes, skin, lungs, heart, and peripheral nerves.

6.1.1.1 Epidemiology

DeÞnite RA has a worldwide prevalence of about 1% of the population (0.3Ð2.1%). The onset is most frequent in the fourth to Þfth decade of life. Women are three times more likely to be affected than men, with 80% of all patients developing the disease between the ages of 35 and 50. Recent data indicate that the incidence of RA may be diminishing. Moreover, disease severity appears to be declining, although it is uncertain whether this reßects more aggressive therapeutic interventions. RA is seen throughout the world and affects all races [1].

Signs and Symptoms of Joint Involvement

Pain, stiffness, swelling, tenderness, and limitation of motion in the involved joints are the characteristic RA complaints, often associated with constitutional symptoms, such as weakness, fatigue, anorexia, and weight loss [2]. Morning stiffness lasting more than 1 h is indicative of joint inßammation; its duration can be used to assess disease activity. Metacarpophalangeal and proximal interphalangeal joints are the joints most commonly involved in RA, often leading to hyperextension of the proximal interphalangeal joints and ßexion of the distal interphalangeal joints (swan-neck deformity), and radial deviation of the wrist and ulnar deviation of the Þngers (Z-deformity) (Fig. 6.1) [3]. Wrist joint involvement can produce limitation of motion, deformity, and compression of the median nerve (carpal tunnel syndrome). Elbow and knee synovial inßammation and proliferation may lead to

Fig. 6.1 Hand and wrist X ray of patient with rheumatoid arthritis. Note the arthritic changes in the metacarpal bones, with both bony destruction and new bone formation and fusion of the articulation between the metacarpals. Note also the bony destruction of the distal intraphalangeal joints, the ulnar deviation, and the formation of the Z deformity

Fig. 6.2 X ray of knee joint of a patient with rheumatoid arthritis. Note the hyperostosis and collapse of the joint space

Fig. 6.3 X ray of foot and ankle of a patient with rheumatoid arthritis. Note the loss of joint spaces between the metatarsal bones

Fig. 6.4 Chest X ray of patient with rheumatoid arthritis

loss of full extension (Fig. 6.2). Ankle and foot arthritis may cause deformities (hallux valgus and plantar subluxation of metatarsal heads) and severe pain during ambulation (Fig. 6.3). Cervical spine osteochondral destruction may lead to atlantoaxial subluxation, which may cause paresthesias in the shoulders or arms during neck movement, pain radiating up into the occiput,

and, less commonly, slowly progressive spastic quadriparesis, often with painless sensory loss of the hands (Fig. 6.4). Other joints that may be affected are the temporomandibular, cricoarytenoid, ossicles of the ear, sternoclavicular, and manubriosternal joints. Hip involvement is uncommon. Thoracic and lumbar spine joint disease cannot be ascribed to RA.