Christelle Darrieutort-Laffite1, Vincent André2, Gilles Hayem3, Alain Saraux4, Véronique Le Guern5, Claire Le Jeunne5, Xavier Puéchal6. 1. National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, hôpital Cochin, université Paris Descartes, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France; Department of Rheumatology, Nantes University Hospital, 44000 Nantes, France. 2. Department of Rheumatology, Le Mans General Hospital, 72000 Le Mans, France. 3. Department of Rheumatology, hôpital Bichat, Assistance publique-Hôpitaux de Paris, 75018 Paris, France. 4. Department of Rheumatology, hôpital de la Cavale Blanche, université de Brest, 29200 Brest, France. 5. National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, hôpital Cochin, université Paris Descartes, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France. 6. National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, hôpital Cochin, université Paris Descartes, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France; Inserm U1016, CNRS UMR 8104, institut Cochin, 75014 Paris, France. Electronic address: xavier.puechal@cch.aphp.fr.
Abstract
OBJECTIVES: To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS). METHODS: Report of three new cases. Only cases fulfilling the American-European consensus criteria for SS and the European Society for the Study of Interstitial Cystitis criteria with positive histological findings for IC were included. RESULTS: Thirteen cases of SS and IC have been reported in women, including the three reported here, with a mean age of 54 years. SS appeared first in 77% (n=10) of cases, a mean of 6.6 years before IC. The symptoms of IC included pollakiuria (n=11), lower abdominal pain (n=8), urinary urgency (n=5), painful micturition (n=6), hematuria (n=3) and dysuria (n=3). Urinary dilatation occurred in three cases, leading to acute renal failure in two patients. The diagnosis of IC was confirmed by anatomical evidence of cystitis inflammation on bladder biopsy in all (n=13) patients. Treatment was reported for nine patients, seven of whom (78%) received corticosteroid treatment, which was partially or completely effective in six cases. Immunosuppressive treatment was added in three cases (cyclosporine, n=2; azathioprine, n=1; cyclophosphamide, n=1). Local bladder treatments were performed, with hydraulic distension in five cases and DMSO instillation in one patient. A urinary catheter was inserted in the two cases of acute obstructive renal failure. CONCLUSIONS: Urinary symptoms without infection should lead the physician to consider a diagnosis of IC in SS patients. Urinary dilatation may occur, leading to acute obstructive renal failure. Corticosteroid treatment may be effective and local treatments have been tried.
OBJECTIVES: To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS). METHODS: Report of three new cases. Only cases fulfilling the American-European consensus criteria for SS and the European Society for the Study of Interstitial Cystitis criteria with positive histological findings for IC were included. RESULTS: Thirteen cases of SS and IC have been reported in women, including the three reported here, with a mean age of 54 years. SS appeared first in 77% (n=10) of cases, a mean of 6.6 years before IC. The symptoms of IC included pollakiuria (n=11), lower abdominal pain (n=8), urinary urgency (n=5), painful micturition (n=6), hematuria (n=3) and dysuria (n=3). Urinary dilatation occurred in three cases, leading to acute renal failure in two patients. The diagnosis of IC was confirmed by anatomical evidence of cystitis inflammation on bladder biopsy in all (n=13) patients. Treatment was reported for nine patients, seven of whom (78%) received corticosteroid treatment, which was partially or completely effective in six cases. Immunosuppressive treatment was added in three cases (cyclosporine, n=2; azathioprine, n=1; cyclophosphamide, n=1). Local bladder treatments were performed, with hydraulic distension in five cases and DMSO instillation in one patient. A urinary catheter was inserted in the two cases of acute obstructive renal failure. CONCLUSIONS: Urinary symptoms without infection should lead the physician to consider a diagnosis of IC in SS patients. Urinary dilatation may occur, leading to acute obstructive renal failure. Corticosteroid treatment may be effective and local treatments have been tried.
Authors: Guillermo Carvajal Alegria; Pierre Gazeau; Sophie Hillion; Claire I Daïen; Divi Y K Cornec Journal: Clin Rev Allergy Immunol Date: 2017-10 Impact factor: 8.667
Authors: Gabriel Maciel; Luisa Servioli; Carlotta Nannini; Alvise Berti; Cynthia S Crowson; Sara J Achenbach; Eric L Matteson; Divi Cornec Journal: RMD Open Date: 2018-02-28