© JAPI • July 2012 • VOl. 60
Sjogren’s Syndrome Presenting with Hypokalemic
Sumita Agrawal*, Vishrant Bharti**, Mayur N Jain*, Prashant D Purkar**, Avinash Verma*,
Alaka K Deshpande***
*PG Resident Doctor, **lecturer, ***Prof. of Medicine, Department of
Medicine, Sir J.J. Group of Hospitals, Byculla, Mumbai
Received: 07.10.2010; Revised: 24.12.2010; Accepted: 13.01.2011
since one day. She was afebrile with a pulse rate of 78/min in
sinus rhythm and BP 120/80 mm/Hg. Within minutes of arrival,
she developed sudden respiratory failure and was intubated.
She was shifted to intensive care unit where respiratory rate was
14/min on ventilator support with IPPV mode. There were no
enlarged lymph nodes or parotid glands or thickened nerves.
8 year female with no significant past and family history was
brought to the emergency ward with sudden onset weakness
She had bilateral symmetrical flaccid quadriplegia with
weakened neck muscles, diminished reflexes and mute plantars.
There was no cranial nerve or sensory involvement. All this was
highly s/o AIDP. However, EMG/ NCV ruled out AIDP.
Serum sodium was 150mEq/l and serum potassium was
2.5 mEq/l. ECG also showed prominent u waves. She was in
metabolic acidosis with pH was 7.085 and a normal anion gap.
liver and Renal functions were normal. Transtubular potassium
gradient (TTKG) = 11 indicated towards Renal Tubular acidosis
Hypokalemia was corrected with i.v. potassium chloride.
On 5th day respiration improved and she was weaned off the
Autoantibody screen revealed positive antinuclear antibody
titer of 1:320 with speckled pattern. Anti dsDNA, anti-Sm
antibodies, rheumatoid factor and anti u1snRNP were negative.
Thyroid function tests were normal. Anti-Ro (SS-A) and anti
-la (SS-B) antibody were strongly positive in very high titer of
214.22 Ru/ml (> 20 is positive) and 81.06 Ru/ml (> 20 is positive)
respectively. These reports and distal RTA raised a high index
of suspicion of Sjogren syndrome (SS).
A salivary gland biopsy demonstrating multiple foci of
lymphocyte and plasma cell infiltrate with ducts revealing
intraepithelial lymphocyte infiltrate further corroborated
Sjogren’s syndrome (Figure 1).
Patient could not afford salivary gland scintigraphy and
sialography. Renal biopsy was refused by patient.
Patient has been maintained on potassium chloride solution,
sodium bicarbonate capsules, low dose prednisolone and is on
regular outpatient follow up.
We report a rare case of a 38 year old female who presented with sudden onset flaccid quadriplegia and respiratory
arrest with no significant past clinical history. She was later found to have hypokalemia due to distal renal tubular
acidosis and further diagnosed as case of Sjogrens Syndrome.
There have been few case reports where hypokalemic periodic
paralysis has been investigated and has led to the diagnosis
of Sjogren Syndrome. The first such case report is as early as
1981.1 India’s first such case was reported in1996 in JAPI by
Thomas et al. from CMC Vellore.2 Rao et al 2006 studied 31 cases
of hypokalemic periodic paralysis where 3 cases had Sjogren
Syndrome.3 This indicates that 10% of people with hypokalemic
periodic paralysis can have Sjogren syndrome which if diagnosed
early has more management options available.
Our patient, an otherwise healthy female, presented with
acute onset flaccid quadriplegia without sensory involvement.
A differential Diagnosis of AIDP and HPP was considered.
Hypokalemia favored the second diagnosis which was
strengthened by immediate improvement of respiratory arrest
by potassium supplementation. Further, metabolic acidosis and
raised TTKG indicated towards Distal RTA.
Distal RTA may be primary or more commonly secondary to
paraproteinaemia, medullary sponge kidney, nephrocalcinosis,
obstructive uropathy and autoimmune disease.4 Hypokalemia
due to dRTA is usually a late manifestation. However it has been
reported in < 2% cases of Sjogren syndrome (SS) as a presenting
manifestation.5 But hypokalemic paralysis with respiratory arrest
as a presenting feature has been documented only in 4 cases.6-9
Our case lacked ocular and oral dryness which may develop
The systemic manifestations necessitate corticosteroids,
cytotoxic agent or both. Our patient is presently receiving low
dose of steroid therapy and is currently asymptomatic.
We report this case to highlight the fact that patients
presenting with HPP can be due to early SS which needs to be
investigated and started on appropriate line of management.
1. Raskin RJ, Tesar JT, lawless OJ Hypokalemic periodic paralysis in
Sjögren’s syndrome. Arch Intern Med 1981;141:1671-3.
2. Thomas N, Ramakrishna B, Seshadri MS. Hypokalemic periodic
paralysis: an unusual cause. J Assoc Physicians India 1996;44:207-8
3. 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.
4. Chan JC, Alon u. Tubular disorders of acid-base and phosphate
metabolism. Nephron 1985;40:257-279.
56 © JAPI • July 2012 • VOl. 60
5. Bossini N, Savoldi S, Franceschini F, et al. Clinical and
morphological features of kidney involvement in primary Sjögren
syndrome. Nephrol Dial Transplant 2001;16:2328-36.
6. Poux JM, Peyronnet P, Le Meur Y, Favereau JP, Charmes JP,
Leroux-Robert C. Hypokalemic quadriplegia and respiratory arrest
revealing primary Sjögren’s syndrome. Clin Nephrol 1992;37:189–
7. Hiroshi Ohtani, Hirokazu Imai, Takahito Kodama, Keiko Hamai,
Atsushi Komatsuda, Hideki Wakui and Akira B. Miura. Severe
hypokalaemia and respiratory arrest due to renal tubular acidosis
in a patient with Sjogren’s syndrome. Nephrol Dial Transplant
8. Fujimoto T, Shiiki H, Takahi y, Dohi K. Primary Sjogren’s syndrome
presenting as hypokalemic periodic paralysis and respiratory arrest.
Clin Rheumatol 2001;20:365-368.
9. Reddy KS, Jha V, Nada R, Kohli HS, Sud K, Gupta Kl, Sakhuja
V. Respiratory paralysis in sjogren’s syndrome with normal renal
function. Natl Med J India 2003;16:253-4.
10. Raskin RJ, Tesar JT, lawless OJ. Hypokalemic periodic paralysis in
Sjögren’s syndrome. Arch Intern Med 1981;141:1671-3.
11. Pun KK, Wong CK, Tsui Ey, Tam SC, Kung AW, Wang CC.
Hypokalemic periodic paralysis due to the Sjögren syndrome in
Chinese patients. Ann Intern Med 1989:110:405-6.
Fig. 1 : Multiple foci of periductal and intra epithelial
lymphoplasma cell infiltrate are seen forming lymphoepithelial