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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2018  |  Volume : 29  |  Issue : 2  |  Page : 440-442
Systemic lupus erythematosus with Sjögren's syndrome and renal tubular acidosis presenting as nephrogenic diabetes insipidus


Division of Internal Medicine, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India

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Date of Web Publication10-Apr-2018
 

   Abstract 

Systemic lupus erythematosus (SLE) presenting as diabetes insipidus (DI) is a rare association; there is a case report of neurogenic DI in patients of SLE; however, SLE and nephrogenic DI has not been reported in literature. We present a case of SLE presenting as nephrogenic DI. We report a case who presented with DI (nephrogenic) and fulfilled criteria for SLE and Sjögren's syndrome with renal tubular acidosis.

How to cite this article:
Parrey AH, Ahmad F, Ahmad M, Basher A. Systemic lupus erythematosus with Sjögren's syndrome and renal tubular acidosis presenting as nephrogenic diabetes insipidus. Saudi J Kidney Dis Transpl 2018;29:440-2

How to cite this URL:
Parrey AH, Ahmad F, Ahmad M, Basher A. Systemic lupus erythematosus with Sjögren's syndrome and renal tubular acidosis presenting as nephrogenic diabetes insipidus. Saudi J Kidney Dis Transpl [serial online] 2018 [cited 2020 Jun 6];29:440-2. Available from: http://www.sjkdt.org/text.asp?2018/29/2/440/229272

   Case Report Top


A 30-year-old female presented with 18-month history of polyuria and polydipsia; there were a history of pain and swelling of hand joints for five months and sicca symptoms. Clinical examination revealed malar rash and active oral ulcers.

The initial evaluation indicated a diagnosis of systemic lupus erythematosus (SLE) with Sjögren's syndrome [Table 1]. Water deprivation test showed a 24-h urinary volume 8 L, [Table 2] and the diagnosis of nephrogenic diabetes insipidus (DI) and distal renal tubular acidosis was made.
Table 1: Blood results.

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Table 2: Vasopressin test.

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   Discussion Top


DI is characterized by polyuria, urine volume in excess of 2 L/m2/24 h. This is a disorder that could be either due to arginine vasopressin (AVP) deficiency (central DI) or AVP resistance which causes nephrogenic DI or excessive water intake (primary polydipsia). The diagnosis of DI is made by water deprivation test. In our patient, water deprivation test revealed that baseline urine osmolality was 103 mOsm/kg. Urine osmolality <300 mOsm/ kg is true of both central as well as nephrogenic DI. After giving vasopressin, the urinary osmolality will rise to more than 750 mOsm/ kg in central DI and primary polydipsia but will have minimal or no increase in urine osmolality in nephrogenic DI. In our case, there was minimal increase in urine osmolality from 117 mOsm/kg to 138 mOsm/kg.

SLE in a chronic autoimmune disorder with multisystem involvement.[1],[2] There have been reports of patient with nephrogenic DI with Sjögren's syndrome[3] and neurogenic DI in patients of SLE lymphocytic infundibuloneurohypophysitis.[3],[4],[5] Our patient fulfilled the American College of Rheumatology criteria for SLE[6] (arthritis, malar rash, oral ulcers, and positive ANA) and American–European consensus criteria for Sjögren's syndrome[7] (oral symptoms, oral signs, ocular symptoms, ocular signs, and positive anti-Ro/La).

The diagnosis of renal tubular acidosis was made in view of metabolic acidosis (serum pH = 7.17), alkaline urine (urine pH = 6.8), and low serum bicarbonate (serum HCO3 = 16.8); in addition, the patient had SLE and Sjögren's syndrome; both of them are considered as etiologic factors of RTA. However, the patient never had hypokalemia. Because the serum pH was already acidic, the need for acid loading test was not needed to induce acidosis.

Conflict of interest: None declared.

 
   References Top

1.
Dubois EL, Tuffanelli DL. Clinical manifestations of systemic lupus erythematosus. Computer analysis of 520 cases. JAMA 1964; 190:104-11.  Back to cited text no. 1
[PUBMED]    
2.
Harvey AM, Shulman LE, Tumulty PA, Conley CL, Schoenrich EH. Systemic lupus erythematosus: Review of the literature and clinical analysis of 138 cases. Medicine (Baltimore) 1954;33:291-437.  Back to cited text no. 2
[PUBMED]    
3.
Sánchez-Román J, Castillo-Palma MJ, Ocaña Medina C, Villamil-Fernández F, Wichmann I. Neurogenic diabetes insipidus in patients with systemic lupus erythematosus. Ann Rheum Dis 1998;57:261-2.  Back to cited text no. 3
    
4.
Shearn MA, Tu WH. Nephrogenic diabetic insipidus and other defects of renal tubular function in Sjoergren's syndrome. Am J Med 1965;39:312-8.  Back to cited text no. 4
[PUBMED]    
5.
Hashimoto K, Asaba K, Tamura K, Takao T, Nakamura T. A case of lymphocytic infundibuloneurohypophysitis associated with systemic lupus erythematosus. Endocr J 2002; 49:605-10.  Back to cited text no. 5
[PUBMED]    
6.
Bartels CM, Muller D. Systemic Lupus Erythematosus (SLE). Available from: http://www.emedicine.medscape.com/article/3 32244-overview.  Back to cited text no. 6
    
7.
Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren's syndrome: A revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61: 554-8.  Back to cited text no. 7
[PUBMED]    

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Correspondence Address:
Dr. Ashaq Hussain Parrey
Division of Internal Medicine, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir
India
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DOI: 10.4103/1319-2442.229272

PMID: 29657217

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