Home | About us | Editorial board | Search | Ahead of print | Current issue | Archives | Submit article | Instructions | Subscribe | Contacts | Advertise | Login 
 
Search Article 
  
Advanced search 
  Users Online: 568 Home Print this page Email this page Small font sizeDefault font sizeIncrease font size  

 
Table of Contents
CASE REPORT WITH REVIEW OF LITERATURE
Year : 2012  |  Volume : 16  |  Issue : 5  |  Page : 853-855

Hypokalemic paralysis as a presenting manifestation of primary Sjögren's syndrome: A report of two cases


1 Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, India
2 Department of Clinical Immunology and Rheumatology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication6-Sep-2012

Correspondence Address:
Rajesh Khadgawat
Associate Professor, Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2230-8210.100684

Rights and Permissions
   Abstract 

Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Overt or latent renal tubular acidosis (RTA), caused by tubulointerstitial nephropathy, is a common extraglandular manifestation of pSS. Hypokalemic paralysis is a well known, albeit rare complication of severe distal RTA from any cause. Cases of pSS manifesting for the first time as hypokalemic paralysis caused by distal RTA have been rarely reported. We herein present our experience of two cases, who presented to us for evaluation of hypokalemic paralysis and on work up found evidence of distal RTA, which on further work up found to be secondary to pSS. A high index of suspicion for pSS should be kept in all patients with hypokalemic paralysis.

Keywords: Hypokalemic paralysis, interstitial nephritis, renal tubular acidosis, Sjφgren′s syndrome


How to cite this article:
Khandelwal D, Bhattacharya S, Khadgawat R, Kaur S, Tandon N, Ammini AC. Hypokalemic paralysis as a presenting manifestation of primary Sjögren's syndrome: A report of two cases. Indian J Endocr Metab 2012;16:853-5

How to cite this URL:
Khandelwal D, Bhattacharya S, Khadgawat R, Kaur S, Tandon N, Ammini AC. Hypokalemic paralysis as a presenting manifestation of primary Sjögren's syndrome: A report of two cases. Indian J Endocr Metab [serial online] 2012 [cited 2020 Apr 7];16:853-5. Available from: http://www.ijem.in/text.asp?2012/16/5/853/100684


   Introduction Top


Sjögren's syndrome is a slowly progressing autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, mainly the lacrimal and salivary glands, resulting in impaired secretory function. Simultaneously, systemic features of cutaneous, respiratory, renal, hepatic, neurologic, and vascular nature often occur. [1] The syndrome can present either alone (as primary Sjögren's syndrome) or in the context of underlying connective tissue disease (as secondary Sjögren's syndrome). [2] Renal involvement is well-recognized extra glandular manifestation of primary Sjögren's syndrome (pSS). Most common manifestations are related to tubular dysfunction resulting from chronic interstitial nephritis and can manifest as distal renal tubular acidosis (RTA), proximal RTA, tubular proteinuria, and nephrogenic diabetes insipidus. [3],[4] Hypokalemic paralysis rarely occurs as the first manifestation of a renal tubule disorder due to pSS. We herein present two cases who presented to us with hypokalemic paralysis, secondary to pSS.


   Case Reports Top


Case 1

A 19-year-old unmarried lady presented to our hospital for evaluation of two episodes of generalized weakness. First episode occurred 4 years back, while second episode occurred 2 months back. Both the episodes were acute in onset, with weakness of all four limbs and neck muscles, and occurred during recovery from febrile illness. There was no sensory, bladder, or bowel involvement during these episodes. She was found to have hypokalemia (documented serum K + of 1.9 and 2.2 meq/L; normal range 3.5-5.5 meq/L) on both the occasions and complete recovery occurred in a span of 48 h on receiving intravenous and oral potassium. She was prescribed oral potassium supplementation. She was referred to endocrinology department for evaluation of hypokalemia. There was no history of fever, joint pain, skin rash, photosensitivity, or parotid swelling. There is no family history of a similar illness. At presentation to us, she had normal general physical and neurological examination. Investigations are summarized in [Table 1].
Table 1: Laboratory parameter of patients


Click here to view


A diagnosis of distal RTA was made in view of an alkaline urinary pH (>5.5) in the setting of metabolic acidosis. There was no evidence of any proximal tubular dysfunction. She was evaluated for etiology of distal RTA, which revealed positive rheumatoid factor (RF) and antinuclear antibody (ANA), while serum C3 level was normal and anti-dsDNA was negative. Schirmer's test was positive in both eyes and her serum anti-Ro and anti-La antibody levels were unequivocally elevated. The buccal mucosal biopsy was consistent with Sjögren's syndrome. She was discharged on oral potassium and alkali supplementation.

Case 2

A 22-year-old married lady presented for evaluation of an episode of acute onset flaccid quadriparesis without any sensory, bladder, or bowel involvement. The episode occurred 15 days before she presented to us. At the time of episode, she was found to have low serum potassium of 1.9 meq/L. She improved within 48 h after receiving potassium supplementation. She had been taking oral potassium supplementation regularly since then and did not develop any further episodes of weakness. There was no history of fever, joint pain, skin rash, photosensitivity, or parotid swelling. There is no family history of a similar illness. Her general and systemic examination including neurological examination was normal. Investigations are summarized in [Table 1].

A diagnosis of distal RTA was made. Bicarbonate loading test ruled out an associated proximal tubular dysfunction. On working up for the etiology of distal RTA, she was found to have elevated titer of RF and ANA, her serum C3 level was normal, and anti-dsDNA was negative. Schirmer's test was positive in both eyes. The mucosal biopsy from lower lip was consistent with Sjögren's syndrome.


   Discussion Top


pSS is a disease of exocrine glands presenting with manifestations related to dry eyes and dry mouth. Nonexocrine organ systems may also be involved, including skin, lung, gastrointestinal tract, central and peripheral nervous system, muscular skeletal apparatus, and the kidney. [1],[5] The reported rate of renal involvement in pSS in literature is variable ranging from 4.2% to 50%. [4] The spectrum of renal disease includes interstitial nephritis, which can manifest as distal RTA, proximal RTA, tubular proteinuria, nephrogenic diabetes insipidus, glomerular diseases, or renal failure. [3],[4] The most common manifestations are related to tubular dysfunction which results from chronic interstitial nephritis. [3] Hypokalemia is the most common electrolyte abnormality in patients with dRTA. The mechanisms of distal RTA-induced hypokalemia include decreased distal tubular Na delivery, secondary hyperaldosteronism, defective H-K ATPase, and bicarbonaturia. [6]

Although hypokalemia is frequent sequel of RTA, a severe symptomatic decrease in serum potassium concentration has been described in a few cases only. Hypokalemic paralysis is a rarely encountered complication of RTA secondary to pSS. Only 18 cases were described between 1966 and 2004. [7] Subsequently, approximately 25 more cases has been reported. [8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21] Here, we present a report of two cases that presented to us with hypokalemic paralysis secondary to distal RTA, which on further work up, was found secondary to pSS. Both of our patients did not have any evidence of proximal tubular dysfunction. In conclusion, the kidney involvement in pSS can uncommonly present as hypokalemic paralysis in the absence of significant sicca symptoms or may precede sicca symptoms. Sjögren's syndrome should be instigated in any patient presenting with hypokalemic paralysis from RTA, even in the absence of the sicca syndrome.

 
   References Top

1.Fox RI. Sjögren's syndrome. Lancet 2005;366:321-31.  Back to cited text no. 1
[PUBMED]    
2.Mavragani CP, Moutsopoulos NM, Moutsopoulos HM. The management of Sjögren's syndrome. Nat Clin Pract Rheumatol 2006;2:252-61.  Back to cited text no. 2
    
3.Goules A, Masouridi S, Tzioufas AG, Ioannidis JP, Skopouli FN, Moutsopoulos HM. Clinically significant and biopsy-documented renal involvement in primary Sjögren's syndrome. Medicine (Baltimore) 2000;79:241-9.  Back to cited text no. 3
    
4.Bossini N, Savoldi S, Franceschini F, Mombelloni S, Baronio M, Cavazzana I, et al. Clinical and morphological features of kidney involvement in primary Sjögren's syndrome. Nephrol Dial Transplant 2001;16:2328-36.  Back to cited text no. 4
    
5.Manthorpe R, Asmussen K, Oxholm P. Primary Sjögren's syndrome: Diagnostic criteria, clinical features and disease activity. J Rheumatol 1997;24 Suppl 50:S8-11.  Back to cited text no. 5
    
6.Lin SH, Cheema-Dhadli S, Gowrishankar M, Marliss EB, Kamel KS, Halperin ML. Control of excretion of potassium: Lessons from studies during prolonged total fasting in human subjects. Am J Physiol 1997;273:F796-800.  Back to cited text no. 6
    
7.Soy M, Pamuk ON, Gerenli M, Celik Y. A primary Sjögren's syndrome patient with distal renal tubular acidosis, who presented with symptoms of hypokalemic periodic paralysis: Report of a case study and review of the literature. Rheumatol Int 2005;26:86-9.  Back to cited text no. 7
    
8.Cheng CJ, Chiu JS, Chen CC, Lin SH. Unusual cause of hypokalemic paralysis in aged men: Sjögren syndrome. South Med J 2005;98:1212-5.  Back to cited text no. 8
    
9.Kawashima M, Amano T, Morita Y, Yamamura M, Makino H. Hypokalemic paralysis and osteomalacia secondary to renal tubular acidosis in a case with primary Sjögren's syndrome. Mod Rheumatol 2006;16:48-51.  Back to cited text no. 9
    
10.El Otmani H, Moutaouakil F, Aghai R, Rafai MA, Bourezgui M, Benkirane A, et al. Symptomatic periodic paralysis secondary to primary Sjogren's syndrome. Rev Neurol (Paris) 2006;162:640-2.  Back to cited text no. 10
    
11.Rao N, John M, Thomas N, Rajaratnam S, Seshadri MS. Aetiological, clinical and metabolic profile of hypokalaemic periodic paralysis in adults: A single-centre experience. Natl Med J India 2006;19:246-9.  Back to cited text no. 11
    
12.Prakash EB, Fernando ME, Sathiyasekaran M, Bhoopathy RM, Jayanth JJ, Samuel J. Primary Sjögren's syndrome presenting with distal renal tubular acidosis and rhabdomyolysis. J Assoc Physicians India 2006;54:949-50.  Back to cited text no. 12
    
13.Moroviæ-Vergles J, Galesiæ K, Vergles D. Primary Sjogren's syndrome presenting as hypokalemic paralysis. Ann Saudi Med 2007;27:125-7.  Back to cited text no. 13
    
14.Ren H, Wang WM, Chen XN, Zhang W, Pan XX, Wang XL, et al. Renal involvement and follow up of 130 patients with primary Sjögren's syndrome. J Rheumatol 2008;35:278-84.  Back to cited text no. 14
    
15.Comer DM, Droogan AG, Young IS, Maxwell AP. Hypokalemic paralysis precipitated by distal renal tubular acidosis secondary to Sjögren's syndrome. Ann Clin Biochem 2008;45:221-5.  Back to cited text no. 15
    
16.Aygen B, Dursun FE, Dogukan A, Ozercan IH, Celiker H. Hypokalemic quadriparesis associated with renal tubular acidosis in a patient with Sjögren's syndrome. Clin Nephrol 2008;69:306-9.  Back to cited text no. 16
    
17.Toy WC, Jasin HE. An unusual case of hypokalemic paralysis associated with primary Sjogren's syndrome. J Ark Med Soc 2008;104:286-7.  Back to cited text no. 17
    
18.Fraer M. A mask and many faces: Hypokalemic periodic paralysis. South Med J 2008;101:887.  Back to cited text no. 18
    
19.Ramachandiran N. Apparently persistent weakness after recurrent hypokalemic paralysis: A tale of two disorders. South Med J 2008;101:940-2.  Back to cited text no. 19
    
20.Baaj M, Safi S, Hassikou H, Tabache F, Mouden K, Hadri L. Hypokalemic paralysis revealing Sjögren's syndrome associated with auto-immune thyroiditis. Nephrol Ther 2010;6:52-6.  Back to cited text no. 20
    
21.Wang CC, Shiang JC, Huang WT, Lin SH. Hypokalemic paralysis as primary presentation of Fanconi syndrome associated with Sjögren syndrome. J Clin Rheumatol 2010;16:178-80.  Back to cited text no. 21
    



 
 
    Tables

  [Table 1]


This article has been cited by
1 Hypokalemic Paralysis due to Primary Sjögren Syndrome: Case Report and Review of the Literature
A. Garza-Alpirez,A. C. Arana-Guajardo,J. A. Esquivel-Valerio,M. A. Villarreal-Alarcón,D. A. Galarza-Delgado
Case Reports in Rheumatology. 2017; 2017: 1
[Pubmed] | [DOI]
2 Hypokalemic Paresis Revealing a Primary Sjögren’s Syndrome
Madiha Mahfoudhi,Hedia Bellali,Imen Gorsane,Mounira El Euch,Sami Turki,Taieb Ben Abdallah
Open Journal of Internal Medicine. 2015; 05(03): 33
[Pubmed] | [DOI]
3 PRIMARY SJOGREN’S SYNDROME AND DISTAL RENAL TUBULAR ACIDOSIS: PRESENTING WITH NEPHROGENIC DIABETES INSIPIDUS SECONDARY TO SEVERE HYPOKALEMIA-A CASE REPORT.
Achouba Singh Ksh,Banashree Devi R. K.,Lokeshwar Singh Kh,Ram Kamei
Journal of Evolution of medical and Dental Sciences. 2013; 2(8): 870
[Pubmed] | [DOI]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Introduction
   Case Reports
   Discussion
    References
    Article Tables

 Article Access Statistics
    Viewed3198    
    Printed54    
    Emailed0    
    PDF Downloaded1086    
    Comments [Add]    
    Cited by others 3    

Recommend this journal