19.4.5Treatment

Management of patients with PBS/IC is difficult and several therapeutic modalities have been tested. Evidence-based recommendations were made after analyzing a large number of studies [70]. The basic therapeutic strategies include medical (oral) treatment, intravesical drug instillation, and surgical intervention. The following drugs reached a high recommendation degree for the treatment of PBS/IC: (a) pentosan polysulfate sodium (PPS), a polysaccharide used to replenish the GAG layer of urothelium; (b) amitriptyline, a tricyclic antidepressant, which can alleviate symptoms of IC probably by blocking the acetylcholine receptors or by inhibiting the reuptake of serotonin and norepinephrine;

(c) hydroxyzine, an H1-receptor antagonist that can block neuronal activation of mast cells by inhibiting serotonin secretion from thalamic mast cells; and

(d) cyclosporine at a dose of 1.5 mg/kg twice daily. Regarding the intravesical instillation, only PPS and dimethyl sulfoxide (DMSO), a chemical solvent that penetrates cell membranes and exhibits analgesic and anti-inflammatory properties, have been found efficacious. Finally, surgical treatment such as transurethral resection and coagulation and major reconstructive surgery are reserved for patients with Hunner’s ulcer or visible lesions.

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Chapter 20

Central Nervous System Involvement

Stanley R. Pillemer, Aaron B. Mendelsohn, and Katrin E. Morgen

20.3Differential Diagnosis with Multiple Sclerosis, Neuromyelitis Optica,

20.1Prevalence and Classification

Neurologic signs and symptoms have been recognized in the setting of Sjögren’s syndrome (SS) since the syndrome was first described by Sjögren in 1935 [1]. In particular, peripheral nervous system involvement in primary SS (pSS) has been well characterized and is believed to affect approximately 10–20% of SS patients [2]. The frequency of central nervous system (CNS) involvement is less clear, however, as controversy exists regarding the frequency and type of CNS manifestations in pSS.

S.R. Pillemer (*)

American Biopharma Corporation, Gaithersburg, MD, USA

A.B. Mendelsohn

School of Health Sciences, Walden University, Minneapolis, MN, USA

K.E. Morgen

Department of Psychiatry, Central Institute of Mental Health (CIMH), Mannheim, Germany