Six weeks prior to presenting at our center, a transthoracic echo (TTE) showed normal left ventricular ejection fraction (LVEF) (60-65%) but the right ventricular systolic pressure (RVSP) was 38 mmHg. She had been diagnosed with pulmonary arterial hypertension (PAH) 6 weeks earlier, and was referred to our center for PAH evaluation. Case 1Ī 45 year-old African American female with seropositive rheumatoid arthritis (RA) for 8 years presented with intractable nausea, headaches, and dizziness to Emory University hospital in December, 2013. A represents case 1 and B represents case 2. Densitometer tracing of protein electrophoresis of patients’ serum. Paraprotein was not detected in either cases.įigure 1. Immunoglobulins G, A and M were elevated in both cases and both cases were negative for cryoglobulins. Both cases had positive rheumatoid factor and cyclic citrullinated peptide antibodies. Plasma viscosity was highest at presentation which was 8.4 for case 1 and 4.3 for case 2. Gamma globulins were higher in case 1 than in case 2 which was paralleled by the plasma viscosity. Figure 1 shows the serum protein electrophoresis at diagnosis of hyperviscosity syndrome for case 1 (Figure 1A) and case 2 (Figure 1B). Resultsīoth patients were African American females. The two cases met the American College of Rheumatology criteria for rheumatoid arthritis. We reviewed charts of two cases of rheumatoid HVS. This study was a prospective non-comparative clinical case series. In plasma cell dyscrasias, serum viscosity of 4-5cp, which corresponds to a serum IgM level of at least 3 g/dL, an IgG level of 4 g/dL, or an IgA level of 6 g/dL, has been associated with development of symptoms. The effectiveness of rituximab in B-cell depletion has been reported in other autoimmune diseases associated with immunoglobulin overproduction such as IgG4-related disease. Rituximab depletes B-cell precursors and mature B cells which express CD20, a cell surface protein, by binding to CD20 and increasing complement-dependent cytotoxicity, inducing antibody-dependent cell-mediated cytotoxicity by binding Fcy receptor on surface of effector immune cells, and by inducing non-classical apoptosis of B cells by crosslinking of multiple CD20 molecules. Rituximab is a mouse:human chimeric IgG1 anti-CD20 antibody which has been approved for treatment of rheumatoid arthritis by the United States Food and Drug Administration based on a large multicenter, randomized, double-blind, placebo-controlled study. Therapy that targets pathogenic B lymphocytes in rheumatoid HVS has not been reported previously. Although the first line of treatment of HVSfrom any cause is plasmapheresis, there are no studies to guide maintenance therapy. Only two cases of pulmonary arterial hypertension (PAH) leading to right heart failure have been reported. Congestive heart failure is uncommon in rheumatoid HVS. Patients usually present with non-specific symptoms such as epistaxis, bleeding gums, headaches, confusion, blurry vision, abdominal pain, fatigue, dyspnea, palmar erythema and stroke. The hallmark of HVS is polyclonal hypergammaglobulinemia associated with elevated serum viscosity leading to impairment of end organ perfusion. Hyperviscosity syndrome (HVS) is a rare extra-articular manifestation of rheumatoid arthritis with only 22 cases reported in literature. Hyperviscosity syndrome, rheumatoid arthritis, pulmonary arterial hypertension Introduction As far as we know, these are the first two cases of rheumatoid HVS which have been treated with rituximab. She was treated with prednisone followed by rituximab infusion. The second case was a post-partum cardiomyopathy which presented with inflammatory polyarthritis and compensated congestive heart failure. The case of pulmonary arterial hypertension was treated with two cycles of plasmapheresis followed by rituximab 1000 mg IV separated by 2 weeks and repeat dosing every 6 months. We report two cases of rheumatoid HVS who presented with either severe pulmonary arterial hypertension or post-partum cardiomyopathy. Rituximab is a powerful B cell depleting therapy which can theoretically eliminate pathogenic B cells and decrease immunoglobulin production in HVS. HVS is characterized by abnormal immunoglobulin production. Although plasmapheresis is effective as the first line treatment in life threatening manifestations such as pulmonary hypertension, stroke, retinal hemorrhages and congestive heart failure, data on effective maintenance immunosuppressive therapy is lacking. Hyperviscosity syndrome (HVS) is a rare manifestation of rheumatoid arthritis with few reported cases.
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