Case Presentation: 68 y/o female presented with symptoms of headache, lightheadedness, visual changes, nausea and fatigue have been ongoing for 7 years but headache specifically has been more persistent over the last few months. She reported the headache to be localized to the left side, temple, behind the eye and sometimes across the front and sometimes in the right temple area. Not pulsating, no aura associated with headache. The headache occurred multiple times every day lasting 15-30 minutes. She gets episodes of lightheadedness and nausea but if she sits and relaxes she will feel better. Erythrocyte Sedimentation Rate (ESR) was measured 79, C-Reactive Protein (CRP) 1. Prednisone 60mg was started in addition to left temporal artery biopsy which showed no histologic evidence of arteritis. Headache did not respond to prednisone, began to occur daily across the front of her head and at night, and intensified in severity since the temporal artery biopsy. Serum protein electrophoresis (SPEP) was done which showed dense M band + monoclonal IgM with hypogammaglobulinemia indicating the diagnosis of Waldenstrom macroglobulinemia. Plasma viscosity was elevated indicative of hyperviscosity syndrome.
Discussion:It is important to differentiate between ESR and CRP and what specifically causes elevation. ESR measures the rate at which erythrocytes settle with gravity in an upright tube of anticoagulated whole blood. ESR is dictated by characteristics of the erythrocytes themselves (size, shape, surface charge) and by the presence of specific plasma proteins. Such plasma proteins include several acute phase reactants (especially fibrinogen) produced by the liver in response to proinflammatory cytokines arising in rheumatologic, infectious, or malignant conditions. These plasma proteins alter the normal repulsive forces (that is, neutralize surface charge) between erythrocytes and promote their ability to aggregate and form rouleaux and sediment more quickly. Some noninflammatory conditions (kidney disease, diabetes mellitus, pregnancy, and obesity) are also associated with elevated fibrinogen levels and can produce an elevated ESR. Some patients make paraproteins (for example, patients with multiple myeloma) that can also cause ESR elevations. CRP is an acute phase reactant synthesized by the liver during inflammation in response to proinflammatory cytokines. CRP can adhere to bacteria and activate complement, promoting phagocytosis. CRP responds rapidly to inflammation, both rising and falling more quickly than ESR. CRP is more stable and less affected by other serum components compared with ESR. When there is wide dissociation between the ESR and CRP with a highly elevated ESR, a monoclonal protein should be suspected. In Temporal Arteritis, neither the ESR nor the CRP is a specific biomarker but due to the potential severity of disease, the patient was treated with prednisone until temporal artery biopsy returned negative and another disease was diagnosed.
Conclusions:In elderly patients with a temporal headache and elevated ESR, though temporal arteritis has to be a consideration, other diagnoses such as monoclonal gammopathies should be screened for when there is wide dissociation between the ESR and CRP.