Normochromic and normocytic anemia (due to retarded erythropoiesis), neutropenia, lymphocytopenia, and thrombocytopenia are frequent Þndings. A hemolytic anemia with reticulocytosis and low hematocrit, with or without positive CoombsÕ test, may antedate other manifestations of the disease by many years. Elevation of the ESR is common in active SLE [189].

It is useful to follow tests that indicate the status of organ involvement known to be present during SLE ßares. These might include hemoglobin levels, platelet counts, urinalysis, and serum levels of creatinine or albumin. There is great interest in identiÞcation of additional biomarkers of disease activity. Candidates include levels of anti-DNA antibodies, several components of complement (C3 is most widely available), activated complement products (including those that bind to the C4d receptor on erythrocytes), IFNinducible genes, soluble IL-2, and urinary adiponectin or monocyte chemotactic protein 1. None is uniformly agreed upon as a reliable indicator of ßare or of response to therapeutic interventions. The physician should determine for each patient whether certain laboratory test changes predict ßare. If so, altering therapy in response to these changes has been shown to prevent ßares.

6.1.2.5 Diagnosis

There is no clinical or laboratory abnormality that is pathognomonic for SLE. Because SLE patients show marked variability in their clinical manifestations and laboratory Þndings, a group of several criteria has been developed in an attempt to include SLE patients and to exclude patients with other disorders and thereby establish a diagnosis of SLE. The American Rheumatism Association developed in 1971 and revised in 1982 a set of 11 diagnostic criteria for the diagnosis of SLE (Table 6.6). A diagnosis of SLE may be made in the presence of four or more of these criteria, serially or simultaneously, during any interval of observation. Our experience, extending over the past 20 years, with a large number of patients with scleritis, episcleritis, anterior uveitis, and retinal vasculitis, shows unequivocally that patients who eventually fulÞl

the criteria for the diagnosis of SLE may fall short of satisfying the criteria for many years while experiencing their recurrent ocular inßammatory problem. Two or three of the established SLE diagnostic criteria may be present (most often ANA positivity, episodic mouth sores, photosensitivity, anemia, or arthritis) coincident with or prior to the onset of the ocular inßammation. Over the ensuing years, other SLE manifestations, such as typical rash, glomerulonephritis, or CNS vasculitis, appear, allowing deÞnitive establishment of the diagnosis. We believe that ocular inßammation (scleritis, episcleritis, anterior uveitis, or retinal vasculitis) should be added to the SLE diagnostic criteria list. We do not know whether or not earlier deÞnitive diagnosis establishment with earlier systemic therapy would favorably alter the prognosis for SLE patients; that possibility can be answered only by a large, prospective controlled clinical trial.

6.1.3Ankylosing Spondylitis

Ankylosing spondylitis (BekhterevÕs disease, MarieÐStrŸmpell disease, rheumatoid spondylitis) is a chronic inßammatory systemic disease of unknown cause that primarily involves the joints of the spine, sacroiliac joints, and periarticular soft tissues. The disease is the prototype of a group of disorders referred to as spondyloarthropathies, and characterized by common features that differentiate them from RA. These features include (1) radiographic sacroiliitis with or without accompanying spondylitis, with a marked tendency toward calciÞcation and ossiÞcation of ligaments (ankylosis); (2) inßammatory peripheral arthritis, often asymmetric, with lack of rheumatoid nodules; (3) no association with rheumatoid factor or ANA; (4) strong association with HLA-B27; (5) tendency for ocular inßammation (especially anterior uveitis and conjunctivitis); (6) variable mucocutaneous lesions; and

(7) occasional cardiac abnormalities. The spondyloarthropaties include AS, ReA, psoriatic arthritis (PA), and enteropathic (IBD) arthritis (Table 6.7) [226].