A 28-year-old mestizo man with a 3-year history of inflammatory
arthritis was admitted to our hospital. An overlap of
rheumatoid arthritis with
systemic lupus erythematosus was suspected; therefore
methotrexate was initiated, and later changed to
leflunomide because of liver toxicity. Prothrombin time, international normalized ratio and activated partial
thromboplastin times were normal (11/10.4 seconds; 1.2; 31.1/26.9 seconds, respectively),
von Willebrand factor activity was observed with low
ristocetin cofactor at 33.6UI/dL, high
von Willebrand factor antigen >200UI/dL, and a low
von Willebrand factor:
ristocetin cofactor to
von Willebrand factor antigen ratio. He was admitted to the emergency room with a 24-hour evolution of progressive
dyspnea,
cough, thoracic
pain, and palpitations, 104 beats/min, 60/40 mmHg, temperature of 38°C, pulse oximetric saturation 88% and 30 breaths/minute. Cold, pale and mottled skin was also observed. He was then transferred to the intensive care unit. The placement of a pulmonary artery
catheter was made. The initial patterns showed a precapillary
pulmonary hypertension; acute
pulmonary embolism was the first choice for diagnosis. Pulmonary angiography was conducted, and when no clot was discovered, pulmonary artery
hypertension associated with
connective tissue disease was considered.
Serum protein electrophoresis confirmed the presence of a massive polyclonal
hypergammaglobulinemia, and no
paraproteinemia or monoclonal cell population was found from the electrophoretic pattern of the patient's plasma.
Hypergammaglobulinemia was the cause of hyperviscosity syndrome associated with
autoantibodies. Three sessions of
plasma exchange therapy were made, and clinical improvement was observed. He was then discharged from the intensive care unit and hospital, respectively. He is now attended by an external consult and has no respiratory symptomatology.
CONCLUSIONS: