Response of Sjogren-related Small Fiber Neuropathy to rituximab

Abstract

Objective Small fiber neuropathy typically presents as loss of temperature and pain sensation and may be associated with other autonomic symptoms. Sjogren's-related ganglionopathy can selectively affect small fibers. Current treatment options for small fiber neuropathy secondary to Sjogren's syndrome include glucocorticoids, IVIG, or immunomodulatory agents such as rituximab. However, these treatments are of uncertain effectiveness and can have variable responses. We present a case of Sjogren's-related small fiber neuropathy that had complete symptom resolution with rituximab. Background A 54 year old female presented to the clinic with neuropathic pain starting in her legs, which ascended to her trunk and arms after one week. She also reported five years of dry eyes. On initial presentation, physical exam showed normal sensation but a diagnosis of small fiber neuropathy was made by skin biopsy. The neuropathy was attributed to seronegative Sjogren's syndrome diagnosed by salivary gland biopsy. She had previously failed treatment with prednisone 40 mg daily and IVIG. After presentation to the clinic, she was started on rituximab every 4 months. A log of symptoms kept by the patient clearly demonstrated a reduction in symptoms following each Rituxan dose. She achieved complete symptom resolution after 19 months with Rituxan 10 mg/mL every 4 months. Design/Methods NA. Results NA. Conclusions In summary, our patient responded dramatically to rituximab after failing treatment with glucocorticoids and IVIG. Treatment of Sjogren's-related small fiber neuropathy has consisted of empiric trials of glucocorticoids and IVIG, although response to these treatments has been variable. Rituximab has been useful in Sjogren's-related neuropathy, although it remains unclear whether it is effective in small fiber neuropathy. Our case illustrates that patients with Sjogren's-related small fiber neuropathy, even if seronegative, may respond dramatically to rituximab. We concluded that rituximab can be considered in treatment of this condition, especially in patients who don't respond to first line immunotherapies.