Cryoglobulinaemic Vasculitis: A Rare Manifestation of Long-standing Rheumatoid Arthritis
Fatemah Baron, Neasa Conneally, Bernadette Lynch
Department of Rheumatology, University College Hospital Galway (UCHG)
Several studies have disclosed a relationship between clinical evidence of vasculitis and the presence of cryoglobulinemia in a Rheumatoid Arthritis (RA) cohort. Cryoglobulinemic vasculitis is characterised by the depositions of cryoglobulins in tissues, which precipitate at low temperatures and redissolve with rewarming. This condition is associated with a variety of disorders including malignancy, infection and autoimmune diseases. The spectrum of manifestations ranges from mild to severe disease with skin being commonly affected.
56-year old Caucasian, male patient, smoker - with a past history of atrial fibrillation and severe erosive seropositive RA presented with a 2-week history of lower limb pain, intermittent numbness, and progressive dusky discolouration of toes associated with pain and numbness. On examination, he was found to have chronic RA changes of the hands and bilateral rheumatoid nodules at the elbows, active synovitis of the small joints of the hands, wrists and ankles and purple discolouration of toes. Peripheral pulses and capillary refill were normal. The remainder of the examination was unremarkable. His workup was negative for any thromboembolic cause, with normal ECHO and CT peripheral angiogram showing three-vessel runoff to the ankles bilaterally and no significant stenosis or dissection. He was found to have raised raised IgA, IgG, IgM and type III cyroglobulinaemia. This clinical picture of chronic seropositive RA with rheumatoid nodules and polyclonal cryoglobulinemia in a smoker led to the diagnosis of cryoglobulinemic vasculitis. Treatment consisted of high dose steroid, DMARD and rituximab. Clinically he made slow recovery but now is improved.
The mean duration between the diagnosis of RA and the onset of vasculitis is 10-14 years. Prevalence is now decreasing with availability of biological therapy. Treatment of Cryoglobulinemic vasculitis is with glucocorticoids and immunosuppressive therapy. Rituximab was reported to be an effective therapy in patients with mixed cryoglobulinemia syndrome not associated with chronic HCV infection. To the best of our knowledge, there are no randomised trials directly comparing rituximab with cyclophosphamide in these patients. Thus, cyclophosphamide should still be considered a therapeutic option in patients with mixed cryoglobulinemia, especially in life-threatening situations.