Rheumatology International

, Volume 38, Issue 12, pp 2251–2262 | Cite as

Renal involvement in primary Sjogren’s syndrome: a prospective cohort study

  • Ankit Jain
  • Bheemanathi Hanuman Srinivas
  • Dantis Emmanuel
  • Vikramraj K. Jain
  • Sreejith Parameshwaran
  • Vir Singh NegiEmail author
Cohort Studies


The objective of the study is to prospectively evaluate the spectrum of clinical and subclinical renal involvement in patients with primary Sjogren’s syndrome (pSS). Of the 174 patients screened, seventy patients with pSS underwent renal function tests, urine examination, renal ultrasound, arterial blood gases, urine pH followed by urine acidification test and renal biopsy (if indicated). Renal tubular acidosis (RTA) was treated with alkali replacement and moderate–severe tubulointerstitial nephritis (TIN) was treated with oral prednisolone. Sixty-two patients completed 1-year follow-up. A comparison was made between patients with and without renal involvement. Thirty-five (50%) patients had renal involvement. They had a lower baseline eGFR (71.85 ± 18.04 vs. 83.8 ± 17, p = 0.005). Twenty-nine patients had RTA (25 complete and 4 incomplete). Eleven patients had urinary abnormalities. Patients with RTA (n = 29) were younger (34.9 ± 9 vs. 42 ± 11.3, p = 0.006), had fewer articular (34% vs. 78%, p = 0.001) and ocular sicca (62% vs. 88%, P = 0.01) than those without RTA (n = 41) and commonly presented with hypokalemic paralysis. On biopsy, TIN (9/17) and IgA nephropathy (3/17) were most common. On follow-up, there was no clinically significant change in eGFR; however, one patient with renal calculi and incomplete distal renal tubular acidosis (dRTA) progressed to complete dRTA. Two patients treated with steroids had marginal improvement in eGFR. Renal involvement in pSS is under-recognized with the most common manifestation being RTA presenting with hypokalemic paralysis. These patients are younger with less articular and sicca symptoms. Subclinical RTA may progress to complete RTA. Renal biopsy should be considered in all patients with renal involvement.


Primary Sjogren’s syndrome Tubulointerstitial nephritis Sicca Renal tubular acidosis Renal biopsy Hypokalemic paralysis 



Primary Sjogren’s syndrome


Renal tubular acidosis


Tubulointerstitial nephritis


Estimated glomerular filtration rate


Distal renal tubular acidosis




Interstitial nephritis


Incomplete distal renal tubular acidosis


American–European consensus group


Antinuclear antibody


Enzyme-linked immunosorbent assay


Rheumatoid factor


Arterial blood gases


Urine acidification test


Complete distal renal tubular acidosis


EULAR Sjögren’s Syndrome Disease Activity Index


Non-steroidal anti-inflammatory drugs


Moderate interstitial fibrosis and tubular atrophy


Focal segmental glomerulosclerosis


Acute tubular necrosis


Neutrophil/lymphocyte ratio


NaCl cotransporter


Carbonic anhydrase


Chronic interstitial nephritis


Toll-like receptors


Statistical package for the social sciences


Sjogren’s syndrome-related antigen A


Sjogren’s syndrome-related antigen B



The authors thank Dr. Gunjan Kumar, Senior resident PSM, for statistical analysis.

Author contributions

(1) The conception and design of the study—AJ, SBH, SP, and VSN. Acquisition of data—AJ, SBH, DE, and VKJ. Analysis and interpretation of data—AJ, SBH, DE, and VKJ. (2) Drafting the article—AJ, DE, and VKJ. Revising it critically for important intellectual content—SBH, SP, and VSN. (3) Final approval of the version to be submitted—AJ, SBH, DE, VKJ, SP, and VSN. (4) Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved—AJ, SBH, DE, VKJ, SP, and VSN.


This study was funded by Intramural Research fund, Jawaharlal Institute of Postgraduate Medicine and Research (Grant number: JIP/Res/Intra-DM, M.Ch/01/2015-16 and JIP/Res/Intra-DM, M.Ch/phas1/grant2/01/2016-17).

Compliance with ethical standards

Conflict of interest

None of the authors have any conflict of interest to declare.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Approved by the Institute Ethics Committee of Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India, held on 11th December, 2014 (JIP/IEC/2014/8/441).

Informed consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

296_2018_4118_MOESM1_ESM.docx (19 kb)
Supplementary material 1 (DOCX 18 KB)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Clinical ImmunologyJawaharlal Institute of Postgraduate Medical Education and Research (JIPMER)PuducherryIndia
  2. 2.Department of PathologyJawaharlal Institute of Postgraduate Medical Education and Research (JIPMER)PuducherryIndia
  3. 3.Department of NephrologyJawaharlal Institute of Postgraduate Medical Education and Research (JIPMER)PuducherryIndia
  4. 4.Immunology and Rheumatology ClinicMeerutIndia
  5. 5.Rajagiri HospitalAluvaIndia
  6. 6.Bhagwan Mahavir Jain HospitalBangaloreIndia

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