Sjogren’s syndrome (SS) is a multi-systemic autoimmune disorder characterized especially by lymphocytic infiltration of exocrine glands. Renal involvement in SS is commonly manifested by tubular dysfunction. A 35 years old female presented to the emergency department with weakness of all four limbs. The baseline investigations in Emergency Department (ED) revealed hypokalemic normal anion gap metabolic acidosis. On further questioning there was history of dryness of mouth, eyes and multiple dental extractions for the last two years. Clinical and biochemical assessment confirmed a diagnosis of SS with type 1 Renal Tubular Acidosis (RTA). Hypokalemic quadriparesis associated with SS is mainly due to type 1 RTA and it can precede the sicca symptoms. We hereby report a case of primary SS with long standing sicca symptoms in which the diagnosis of SS was suspected only after the hypokalemic paralysis. The case highlights the significance of presentation of acute hypokalemia in the ED as it can unmask SS even if it is associated with sicca symptoms. Hypokalemic paralysis if associated with normal anion gap metabolic acidosis should prompt towards diagnosis of SS.
Keywords: Hypokalemia, renal tubular acidosis, Sjogren`s syndrome
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Naik M, Najar MS, Bhat T, Shah Y. Hypokalemic Quadriparesis in a female-The marker for Sjogren’s syndrome. Ann Trop Med Public Health 2013;6:312-4
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Naik M, Najar MS, Bhat T, Shah Y. Hypokalemic Quadriparesis in a female-The marker for Sjogren’s syndrome. Ann Trop Med Public Health [serial online] 2013 [cited 2020 Sep 22];6:312-4. Available from: https://www.atmph.org/text.asp?2013/6/3/312/120992
SS is a chronic slowly progressive autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands of the body, eyes and salivary glands, leading to dry eyes and xerostomia. It is a multi-systemic disease also involving non-exocrine organ systems like lungs, kidneys, skin, blood vessels and muscle, that can lead to lymphomas in 5% of cases.  Renal involvement in SS is commonly as tubulointerstitial nephritis with defects in tubular function. ,,, We report an un-recognized case of primary SS which has been symptomatic for the last two years with sicca symptoms and was diagnosed only after the patient presented as hypokalemic paralysis reflecting the smoldering course of SS and the need of further evaluation of patients presenting as hypokalemic paralysis for SS.
A 35 years old female nor-motensive, non-diabetic, Para 3 presented to ED as sudden onset quadriparesis, without any prior history of fever, drug intake, or insect bite. She had history of dental caries and multiple tooth extractions for last three years. She also had dryness of mouth and eyes of one year duration for which she was following an ophthalmologist who had put her on artificial tears 4 times a day.
On examination her pulse was 84/min, BP 130/70 mm/Hg, orodental hygiene poor, and congested palpebral conjunctiva. Systemic examination was normal. Hb: 10.2 gm/dl, TLC: 4.76×10 9 /l, N 43% L 25% M 10% E 9 %, PLT 226×10 9 /l, ESR 26/1 st hour.
Blood sugar: 117 mg/dl, renal and liver function: normal, urea 23 mg/dl, creatinine: 0.86 mg/dl, bil: 1.02 mg/dl, AST: 39 U/L, ALT: 41 U/L, ALP: 371 U/L, LDH: 410 U/L, amylase: 41 U/L, total protein 8.4 mg/dl, albumin: 3.6 mg/dl, urine examination: normal, ECG: normal, CXR: normal. USG (ultrasound): normal.
ABG-pH: 7.26, HCO 3: 16.6 mMOL/L, Cl: 111 mMOL/l, Na: 137.5 mMOL/L, K: 1.86 mMOL/L, p CO 2: 36 mm Hg, p O 2: 76 mm Hg. Urinary pH: 6.0 On further investigations ANA was positive, Anti ds-DNA was negative, Anti-Ro SS(A) and Anti La SS(B) were positive. The Schirmer`s test was also positive.
The diagnosis of SS was established as our patient fulfilled four out of the six criteria’s of the revised international classification criteria for SS i.e. ocular symptoms, ocular signs, oral symptoms and positive antibody. The patient’s symptom improved dramatically after correcting serum potassium and patient was discharged on potassium supplements and oral sodium bicarbonate tablets.
The prevalence of renal involvement in primary SS varies from 16-67%. ,,,,,,,, Hypokalemic paralysis due to type 1 renal tubular acidosis in SS has been reported worldwide. ,,,,,,,, It may precede the sicca symptoms or it may be the first presentation of SS despite long duration of exocrine gland involvement. ,,,,,,,, The overall incidence of type 1 RTA in SS varies from 5-33 %. , However the defect in urinary acidification has been found to up to 50% in a series of twelve cases by Talal et al. and up to 67% in a series of twenty seven cases by Eriksson et al.and interestingly renal tubular acidosis was the initial manifestation of primary SS in 75 % in a series of 8 cases by Chen et al.,,
The overall prevalence of SS in India is very less as compared to western countries. Porkodi et al. reported that 36 patient of SS were diagnosed over a period of four and half year out of 8000 patients seen with musculoskeletal symptoms amounting to 0.0045 % from southern India.  In another report from northern India only 26 cases of SS were described over a period of ten years.  The lower prevalence can be due to either there is a lack of awareness of this entity in our country by physicians, ophthalmologists and dentists or due to the unrecognized sub clinical sicca symptoms in patients.
The duration of disease in SS at the time of presentation can be longer in patients with renal tubular acidosis as compared to those without renal tubular acidosis. , However in another study it was found that disease duration in patients of SS was shorter with renal involvement than in those with normal acidification capacity.  Therefore, in a busy ED a case of hypokalemic paralysis can simply be a marker of a decade old sub -clinical SS. Hence, evaluating hypokalemia for SS especially in women is recommended.
Case detection and diagnosis are not always clear and consistent in SS, and reporting from our part of world is incomplete and irregular. Hence, awareness of varied forms of presentation of this gradually progressive disorder should be encouraged.
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