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Connective tissue diseases (CTDs) are characterized by immune system dysregulation, circulating autoantibodies and multisystem disease. Diagnosis is made using both clinical and serological features. Renal involvement—manifesting as renal failure or urine dipstick abnormalities—may be the presenting feature of CTD. Renal and general physicians need to have a good understanding of how serological testing can help in the diagnosis of CTD. Here we consider how anti-nuclear antibody (ANA) and extractable nuclear antigen (ENA) testing can aid the diagnosis of CTD in patients with renal disease. We review how these assays are performed and the principles of serological testing. We summarize the clinical features, renal pathology and diagnostic performance of ANA and ENA testing in a range of CTDs. (We do not discuss lupus nephritis, which is comprehensively covered elsewhere in the literature.)

Diagnosing renal involvement in connective tissue disease: interpretation of anti-nuclear autoantibody tests