Common manifestations of Cogans syndrome
Systemic vasculitis occurs in 15% to 20%, usually affecting the large- and medium-sized vessels. Involvement of the aorta and its major branches is well described and typically manifests in a Takayasu-like manner. Musculoskeletal complaints are seen in up to one-third of patients and include arthralgias, myalgias, and inflammatory arthritis. Constitutional symptoms occur in 50%, mostly when systemic disease is present. Other organ involvement includes gastrointestinal (pain, bleeding, hepatomegaly), cardiac (pericarditis, aortic insufficiency), pulmonary (pleuritis), neurologic (headache, peripheral neuropathy, mononeuritis multiplex, meningitis), dermatologic (nodules, rash), and lymphatic (lymphadenopathy, splenomegaly).
Laboratory findings suggestive of inflammation including elevated CRP and ESR, leukocytosis, thrombocytosis, and anemia may be present. ANCA, ANA, RF, APS tests, and complement levels are usually absent or normal.