A Case Of Cryoglobulinemia in a Sjogren’s Patient
Dr. Brona Dinneen, Prof. Paul O’Connell
Rheumatology Department-Beaumont Hospital
We present a case of hypocomplementemia, leukocytoclastic vasculitis and rapidly worsening renal function in a woman with a very recent family history of a death from a vasculitic autoimmune illness with a background of hypocomplementemia and a new diagnosis of transverse myelitis in her daughter.
A 71-year-old woman with a 30-year Hx of Sjogren’s/MCTD disease suddenly developed a purpuric rash on her lower limbs and low C4. Biopsy of the skin was consistent with leukocytoclastic vasculitis. Patients symptoms responded well to prednisolone however would return when decreased to 20mg daily. Repeat bloods showed, negative ANCA, Negative ANF & ENA with normal FBC and urine ACR. CCP and RF were negative. Cryoglobulins were sent and patient was commenced on Cellcept 500mg BD. Patient responded well symptomatically to MMF and steroids were tapered to 10mg with no return of cutaneous symptoms. Her Cryoglobulins were shown to be positive in the first sample.
When patient reached 5mg of Prednisolone her lower limb rash returned, she presented to clinic with a blood pressure of 200/113, periorbital, upper and lower limb oedema and new SOB on exertion.
CXR showed new bilateral pleural effusion, Creatinine 120(baseline 70), PCR-130mg/100ml and severe hypocomplementemia. FBC showed lymphopenia. Echo, HRCT, RHC and CT-AP– all normal. Hepatitis serology continued to be negative. Persistent temperature spikes between 38-39 C o- were treated with IV diuretics, antibiotics and,IVIG with no response, Cellcept was held although no infective source was found.
Renal biopsy performed when clinically improved showed cryoglobulinemia and patient was commenced on 60mg of prednisolone and 1g of rituximab was given 2 weeks apart.
With slow taper of steroids and close follow up – patient continued to improve clinically with renal function and compliments returning to normal. Further Rituximab will be considered at 6 months
Cryoglobulinemic vasculitis occurs when immune complexes containing cryoglobulins effect the small and medium vessels. Although mainly associated with HCV infection, here we see an interesting case of acute deterioration secondary to connective tissue disease on the background of a strong family history. Hereditary complement studies are pending