Arthritic, Metabolic, and Vascular Disorders

Still’s Disease (Adult Onset)

Still’s disease is a seronegative polyarthritis that usually affects young adults. It is characterized in its initial manifestation as a spiking fever and a red/salmon-colored rash, usually over the trunk and extremities. The rash is transient and appears at the time of the fever spikes. The inflammatory arthritis is a polyarthritis or oligoarthritis. It commonly affects the proximal interphalangeal (PIP) and metacarpophalangeal (MCP) joints, as well as the wrists, knees, hips, and shoulders. Occasionally, the cervical spine, intertarsal joints, temporomandibular joints (TMJ), and the distal interphalangeal (DIP) joints are affected. It may lead to fusion of the carpal-metacarpal and the intercarpal joints. Laboratory evaluation commonly shows an elevated white blood cell count as well as an elevated erythrocyte sedimentation rate (ESR). Anemia of chronic disease is commonly present. Secondary nonmusculoskeletal findings include lymphadenopathy, hepatosplenomegaly, pericarditis, and carditis. The disease is treated with nonsteroidal antiinflammatory medications (mild disease). Often, oral corticosteroids are required to control moderate disease along with biologic/nonbiologic disease modifying antirheumatic drugs (DMARDs) for severe disease. Overall, Still’s disease has a good prognosis.

Ankylosing Spondylitis

Ankylosing spondylitis is an insidious-onset seronegative inflammatory condition affecting young individuals, that is, generally younger than 40 years old. It has a uniform sex distribution, but the disease seems to be milder in females. Also, females have more peripheral involvement rather than spine involvement. Ankylosing spondylitis affects the sacroiliac (SI) joints, followed by the spine and peripheral joints. There usually is symmetric loss of spine movement. The peripheral joint involvement occurs in 20% to 30% of ankylosing spondylitis patients and has a predilection for the lower extremities. Achilles’ tendinitis, plantar fascitis, and costochondritis also are associated with the disease process. It is common to have fatigue, weight loss, low-grade fever, and, in more severe cases, uveitis, pulmonary fibrosis, and cardiac abnormalities. Laboratory findings include an elevated ESR. The natural history of ankylosing spondylitis is poorly defined, with some patients experiencing minimal disease and some patients experiencing severe disease. Treatment usually involves physical therapy and antiinflammatories. Refractory cases may respond to antitumor necrosis factor (TNF) agents.

Reiter’s Syndrome

Reiter’s syndrome involves the triad of arthritis, uveitis, and conjunctivitis. It commonly occurs following an episode of either genitourinary or gastrointestinal (GI) infection. It has associated features of inflammatory eye lesions, balanitis, oral ulcers, and keratodermatitis. Reiter’s syndrome has a male-to-female occurrence of 5:1. The arthritis experienced in Reiter’s syndrome is a reactive arthritis rather than an infectious arthritis. It usually occurs 2 to 6 weeks following the onset of an infectious episode. It is asymmetric and mainly affects knees and ankles. It is usually of acute onset. There may be diffuse swelling of fingers and toes, that is, sausage digits. There is commonly inflammatory change at both the Achilles’ tendon insertion and the plantar fascial origin. There also may be associated low-back pain with involvement of the SI joints, making it difficult to distinguish it at times from ankylosing spondylitis. The conjunctivitis in Reiter’s syndrome may be either unilateral or bilateral. It usually is mild and transient and is a noninfectious source like the arthritis. Common skin lesions in Reiter’s syndrome are small, shallow, painless penile ulcers called balanitis circinata. Another associated skin lesion is keratoderma blenorrhagica, which represents hyperkeratotic skin lesions mainly involving the soles of the feet, but they also can be found on the palms and the scrotum. Radiographic findings may demonstrate erosions or periosteal changes, particularly at the Achilles’ tendon insertion or plantar fascial origin. Also, an asymmetric sacroiliitis may be present that is in contrast to the symmetric involvement of ankylosing spondylitis. Reiter’s syndrome also is seronegative but usually demonstrates an elevated ESR and white blood count. Treatment for Reiter’s syndrome involves antiinflammatory medications and intra-articular steroid injections as well as physical therapy. Systemic oral steroids have been shown to be of minimal benefit except in refractory cases. Nonbiologic DMARDs may be necessary. Topical steroids are used for the skin lesions and for the conjunctivitis. The prognosis for Reiter’s syndrome is generally good, with typical duration of 3–5 months with either complete remission or little active disease after 6–12 months. However, 15%–20% of patients may develop a chronic persistent arthritis.

Psoriatic Arthritis

Psoriatic arthritis is the combination of psoriasis and inflammatory arthritis. To make a definitive diagnosis of psoriatic arthritis, skin or nail changes of psoriasis must be present at some point in the course of the disease. The arthritic changes can be present before skin changes develop. The joint pattern in psoriatic arthritis is variable but commonly includes a pauciarticular asymmetric arthritis involving the peripheral joints. It is common to have the spine involved in combination with peripheral joints as well as inflammation of tendon and insertion points of tendons, that is, enthesitis. Digits may become sausage like. There often are associated eye changes, including conjunctivitis, iritis, and episcleritis. Psoriatic arthritis has an equal sex distribution and usually has onset in the 30- to 40-year-old age group. Laboratory results are often normal, but some athletes will present with an elevated ESR and/or a normocytic normochromic anemia. Synovial fluid evaluation typically reveals a mild inflammatory process. Radiographs often reveal DIP erosive disease, sacroiliitis, and enthesopathy and/or periostitis. Treatment of psoriatic arthritis involves the use of antiinflammatory medications (mild disease), physical therapy, and intra-articular corticosteroids to treat the inflammatory arthritis. Oral glucocorticoids are generally avoided due to the increased risk of developing erythroderma or pustular psoriasis. The focus of treatment, however, involves treating the athlete’s skin lesions. Oral methotrexate (a nonbiologic DMARD) is a common therapeutic choice because it treats both the skin lesions and the arthritis. Severe disease may require a biologic DMARD or TNF agent. Psoriatic arthritis was once considered a mild disease with a good prognosis. Now, however, it is considered a more severe disease, and early referral to rheumatology is recommended.

Enteropathic Arthritis

Enteropathic arthritis is arthritis associated with inflammatory GI conditions including ulcerative colitis and Crohn’s disease, and infectious GI conditions, including Shigella, Salmonella, Campylobacter, Yersinia, and Whipple’s disease. The arthritis, when associated with ulcerative colitis or Crohn’s disease, usually is one of a peripheral arthritis with associated sacroiliitis and less often enthesopathies. It often is a transient, oligoarticular, migratory, nondestructive arthritis associated with the bowel disease activity. The knees and ankles are most often involved. Synovial fluid from the affected joints contains mild to severe inflammation. There are a variety of associated cutaneous lesions with the disease, and mucosal, serosal, and ocular lesions may occur. The arthritis with ulcerative colitis and Crohn’s disease often resolves with medical (glucocorticoids or TNF agents) or surgical treatment of the intestinal disease.

The arthritis associated with enteropathic infection often comes on a few weeks following the bowel symptoms. The arthritis, in this case, is a reactive arthritis and, again, affects mainly knees and ankles. There also may be axial joint involvement. Enthesopathies, although not common in association with ulcerative colitis and Crohn’s disease, are common in association with infectious GI conditions and typically involve the plantar fascia and Achilles’ tendon insertions. The arthritis is usually self-limited, resolving weeks to months after the bowel infection. Treatment is symptomatic, involving the use of antiinflammatory medications, physical therapy, and intra-articular corticosteroid injections.