Noninfectious inflammatory conditions

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1. Rheumatoid arthritis. Although usually insidious in onset, RA can present with an acute polyarthritis. Early on, these patients may be seronegative for rheumatoid factor but may have fatigue, anemia, and thrombocytosis. Fever is not commonly seen in RA.

2. Polyarticular JRA may present differently in subsets of children.

a. The younger child has a seronegative oligoarthritis that is often insidious in onset. Serum in approximately 25% will be positive for antinuclear antibodies. A potentially destructive iridocyclitis is seen in this group of children.

b. Another group of children may present with systemic-onset JRA, with high spiking fevers, a transient rash, hepatosplenomegaly, lymphadenopathy, and polyarticular or oligoarticular joint complaints that develop later.

c. The preadolescent girl, in contrast, may present with rheumatoid factor positivity, nodules, and an erosive polyarticular joint disease similar to adult RA (see Chapter25).

3. Systemic lupus erythematosus (SLE) is a classic immune complex disorder that characteristically presents with an RA-like polyarthritis of the small joints of the hands and feet. Marked proliferative synovitis speaks against the diagnosis of SLE. Erosive disease is rare, but the presence of reversible deformity is not. Other clinical features, such as serositis, fever, skin rash, and renal disease, may provide clues to the diagnosis. Laboratory abnormalities can include the presence of serum antinuclear antibodies, anemia, and thrombocytopenia (see Cha.pter..30).

4. Other connective tissue diseases include a spectrum of disorders that produce inflammatory disease of muscles, soft tissues, small blood vessels, and viscera. The initial presentation may include polyarthritis, but more diagnostic features evolve, including Raynaud's phenomenon with digital infarcts, skin thickening, dysphagia, and pulmonary fibrosis suggestive of scleroderma; proximal myopathy and skin rash characteristic of polymyositis/dermatomyositis; and overlap features seen in mixed connective tissue disease.

5. Seronegative spondyloarthropathies. This group of diseases is characterized by presence of the class I histocompatibility antigen HLA-B27; axial arthritis, including spondylitis and sacroiliitis; and inflammatory disease of the eye, skin, and ligamentous insertions (enthesopathy). The joint pattern is usually oligoarthritic and asymmetric, and large joints of the lower extremity are involved. Rheumatoid factor and antinuclear antibodies are not usually found in the serum of these patients. The specific diagnosis is usually defined by the associated clinical features: psoriatic arthritis by the presence of psoriasis, Reiter's syndrome by the concomitant conjunctivitis and urethritis, and enteropathic arthritis by the presence of ulcerative colitis or Crohn's disease.

a. Ankylosing spondylitis has perhaps the least association with peripheral joint involvement of the group; it usually presents insidiously with symptoms in the axial spine. However, inflammatory involvement of the peripheral joints may develop in 25% to 30% of patients at some point in the disease. This most often involves the hips and shoulders (see Chapter.33).

b. Reiter's syndrome typically has a markedly acute presentation of polyarthritis. The triad of conjunctivitis, urethritis, and asymmetric oligoarthritis may not always occur simultaneously, and limited disease has been recognized. Disease onset often follows dysentery or urethritis caused by a number of pathogens. Enthesopathy (inflammation of ligamentous or tendon insertions) and joint inflammation cause the classic "sausage digit" or dactylitis that enlarges the joints and soft tissues of the toes or fingers.

Extraarticular features that are most helpful in diagnosis include circinate balanitis (a psoriasiform rash encircling the glans penis) or a hyperkeratotic rash on the feet (keratoderma blennorrhagicum) (see Cha.pter.36).

c. Psoriatic arthritis can present as asymmetric oligoarticular arthritis of large and small joints; it can also present as a symmetric polyarthritis indistinguishable from RA. Dactylitis, psoriasis, and nail dystrophy are clinical features used to distinguish psoriatic arthritis from RA. Nail involvement, often simple pitting, may be present in 60% of patients with arthritis and psoriasis and is found in only 5% of those with psoriasis alone (see Chapter..35).

d. Enteropathic arthritis usually presents as an asymmetric polyarthritis of the lower extremities, which can predate known inflammatory bowel disease by months to years. It is typically nonerosive, and the peripheral joint inflammation usually responds to therapy of the underlying bowel disease. The axial arthritis may not respond as well. Clues to the diagnosis include abdominal pain, abnormal bowel movements, erythema nodosum, or pyoderma gangrenosum associated with a spondylitic presentation (see Ch.a.pie.L3.4.).

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