Primary Sj gren's Syndrome Presenting with a Huge Pulmonary Mass and Acute Renal Insufficieney.
- Author:
Min Kyu KANG
1
;
Young Sun KOO
;
Pyung Ju HWANG
;
Jong Hak KIM
;
Ki Ryang NA
;
Kwang Sun SUH
;
Kang Wook LEE
;
Young Tai SHIN
Author Information
1. Department of Internal Medicine, Chungnam National University Hospital, Taejon, Korea. kwlee@hanbat.chungnam.ac.kr
- Publication Type:Case Report
- Keywords:
Sj gren's syndrome;
Pseudolymphoma;
ARF;
Cyclophosphamide
- MeSH:
Acute Kidney Injury;
Bronchitis, Chronic;
Cyclophosphamide;
Endocrine Glands;
Gastrointestinal Tract;
Humans;
Kidney;
Lung;
Mouth;
Nervous System;
Pleurisy;
Prednisolone;
Pseudolymphoma;
Pulmonary Fibrosis;
Recurrence;
Salivary Glands;
Skin
- From:Korean Journal of Nephrology
2000;19(4):745-750
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Sj gren's syndrome(SS) is a systemic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, which result in dry eye and dry mouth. Systemic involvement including the lungs, gastrointestinal tract, kidneys, endocrine glands, skin, or nervous system has been reported. Pulmonary abnormalities in SS are lymphoid interstitial infiltration, chronic bronchitis, pulmonary fibrosis and pleurisy. Pulmonary pseudolymphoma associated with acute renal insufficiency is very rare. Recently, we experienced a patient with SS presenting with a huge pulmonary mass in the left upper lobe and acute renal failure. Pulmonary pseudolymphoma and renal functional impairment were completely reversed after a six month treatment with oral prednisolon(1mg/kg, body weight) and monthly cyclophosphamide pulse therapy (12mg/kg, I.V.). There was no evidence of disease recurrence for 16 months after discontinuation of prednisolone therapy.