title: Pure sensory neuropathy in primary Sjögren's syndrome. Longterm prospective followup and review of the literature.
abstract: OBJECTIVE
  To study the clinical course, response to therapy, and longterm outcome of pure sensory neuropathy (PSN) in a series of patients with primary Sjögren's syndrome (SS) followed prospectively in our referral centers.
  
  
  METHODS
  We studied 15 patients (13 women, 2 men) with primary SS and PSN. All patients fulfilled 4 or more of the European diagnostic criteria.
  
  
  RESULTS
  At diagnosis of PSN, clinical manifestations included numbness and paresthesias (11 patients), trigeminal neuropathy (6 patients), and Adie's pupil syndrome (4 patients). In 7 patients, PSN was diagnosed prior to SS, in 5 the diagnoses were made simultaneously, and in the remaining 3 patients PSN was diagnosed after the appearance of SS symptomatology. The mean duration of the prospective PSN followup was 10 years (range 1-20). The progression of PSN was acute in 1 patient (producing severe dysfunction in less than 1 month), subacute in 3 patients, and in the remaining 11, the symptoms progressed slowly over the ensuing years to other extremities. Patients were treated with corticosteroids (n = 13), cyclophosphamide (n = 4), and intravenous immunoglobulins (n = 1), and 2 patients received no treatment. In spite of treatment, most patients showed an indolent and insidious longterm PSN course.
  
  
  CONCLUSIONS
  We found 3 differentiated clinical courses of the PSN in patients with primary SS: subacute progression in less than 1 month (7%), late acceleration of PSN 2-4 years after an initial indolent onset (20%), and a very longterm insidious, chronic evolution (73%). Prospective analysis of the longterm course of PSN shows a chronic and insidious evolution in most patients with PSN and SS, with a poor response to treatment, although stabilization of symptomatology for long periods is often observed.
authors.affiliation: Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain. jfont@clinic.ub.es
authors.forename: Josep
authors.initials: J
authors.lastname: Font
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.forename: Gloria
authors.initials: G
authors.lastname: de la Red
authors.forename: Adolf
authors.initials: A
authors.lastname: Pou
authors.forename: Arnau
authors.initials: A
authors.lastname: Casanova
authors.forename: Mario
authors.initials: M
authors.lastname: García-Carrasco
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
authors.forename: José A
authors.initials: JA
authors.lastname: Molina
authors.forename: Josep
authors.initials: J
authors.lastname: Valls
authors.forename: Albert
authors.initials: A
authors.lastname: Bové
authors.forename: Miguel
authors.initials: M
authors.lastname: Ingelmo
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018501:Antirheumatic Agents / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D011446:Prospective Studies; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D003520:Cyclophosphamide