This document describes a case study of a 33-year-old female patient who presented with acute weakness of all four limbs and was diagnosed with Sjögren's syndrome (SS), distal renal tubular acidosis (dRTA), and hypokalemic quadriparaesis. The patient had a history of dry eyes and mouth for 6 months and persistent hypokalemia for 3 months. Tests showed metabolic acidosis, alkaline urine, hypokalemia, and positive autoantibodies. A salivary gland biopsy found lymphocytic infiltration consistent with SS. The patient was treated with potassium replacement and supplements, and had complete resolution of symptoms within a week. This case highlights the rare occurrence of SS presenting

1. 494 Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4
Address for correspondence:
Dr. Arundhati G. Diwan, Flat No. 16, Laxmi Abhishek, Laxmi Park Colony, Navi Peth, Pune - 411 030, Maharashtra, India.
E-mail: arundhatidiwan@gmail.com
Distal renal tubular acidosis and
quadriparaesis in Sjögren’s syndrome:
A cunning congregate
Arundhati G. Diwan1
, Sachin A. Adukia2
, Shounak V. Annachhatre2
, Yuraj Singh Chowdhury3
1
Professor and Head, 2
Postgraduate student, Department of Medicine, 3
Intern, Bharati Vidyapeeth University Medical College and Bharati Hospital,
Pune, Maharashtra, India
ABSTRACT
Sjögren’s syndrome (SS) is a chronic autoimmune disease, chiefly
affecting the exocrine glandular function of salivary glands and
lacrimal glands. Rarely, it involves the kidneys, central and
peripheral nervous system, muscloskeletal apparatus and lungs.We
report a rare constellation of SS with distal renal tubular acidosis
and quadriparaesis in a young female. History of quadriparaesis
was acute, with rapid progression. Supplementary treatment for
severe hypokalemia was instituted at the earliest, lest the patient
develop respiratory muscle weakness. Concomitantly, metabolic
acidosis with alkaline urine was suspected and subsequently
investigated. Eventually, this was attributed to impaired renal
acidification of urine in the distal tubules. History of dryness of
eyes and mouth since 6 months justified salivary gland biopsy.
The results yielded a lymphocytic infiltrative pathology strongly
favoring SS. The patient benefited from prompt potassium
replacement therapy and had complete resolution over the next
week. Supportive treatment for predictable manifestations was
continued along with potassium supplements.
Keywords: Extraglandular Sjogren’s syndrome, hypokalemic
paralysis, potassium chlorate
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DOI:
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Introduction
Sjögren’s syndrome (SS), also known as the sicca complex,
is a chronic autoimmune disease frequently associated with
xerostomia, keratoconjunctivitis sicca and connective tissue
disorders such as systemic lupus erythematosus, rheumatoid
arthritis or systemic sclerosis. Extraglandular pathology may
involvethekidney(withresultantimpairedurinaryacidification,
chronic kidney disease and tubulointestinal nephritis), brain,
peripheral nerves, liver and lungs.[1-3]
We describe a young
female with acute onset quadriparaesis and hypokalemia who
fully recovered with potassium supplementation.
Case Report
A 33-year-old female presented with acute onset weakness
of all four limbs since 18 h, progressing over the past 4 h.
Initially, she could walk with support, but was unable to
do so at presentation. She gave no history of abdominal
pain, joint pains, hair loss, photosensitivity, recent fever,
urinary symptoms, convulsions or trauma. She gave a
history of dryness of eyes and mouth since 6 months.
There was no history suggestive of bulbar muscle weakness,
hyperthyroidism or myasthenia gravis. She had a past history
of persistent hypokalemia since 3 months, which was treated
outside. However, she was not on any medication at present
nor was she evaluated thus far.
General examination revealed mild pallor and normal vital
parameters. Examination of the central nervous system
revealed hypotonia with grade 3 power, proximally and
distally, in all four limbs. No neck muscle weakness was
present. All deep tendon reflexes were depressed with flexor
plantars.Examinationofthecranialnervesandsensorysystem
was normal. The remainder of the systemic examination was
normal. A diagnosis of hypokalemic periodic paralysis (HPP)
was formulated and investigated. The patient had anemia
(hemoglobin 9.6 gm/dL), hypokalemia (serum potassium 1.8
Case Report
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2. Diwan, et al.: An uncommon cause of quadriparaesis
Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4 495
mEq/L), hypoalbuminemia (serum albumin 2.9 gm/dL) with
hyperglobulinemia (serum globulin 4.0 gm/dL), raised ESR
(54 mm at the end of 1 h), hyperphosphatemia (serum
phosphorus 19.5 mg/dL) and hyperchloremia (serum chloride
110 mEq/L). Other tests for blood urea, serum creatinine,
serum magnesium, corrected serum calcium and thyroid
function tests were normal. Electrocardiogram showed U
waves.Arterialbloodgasanalysissuggestedmetabolicacidosis
(pH 7.29, pCO2
29.4 mmHg, HCO3
13 mmol/L). The urinary
pH was 6.1 and the CT scan of kidney and urinary bladder
(CT-KUB) was normal. The ammonium chloride loading
test was deferred in view of highly alkaline urine despite
metabolic acidosis. The anti nuclear antibody (ANA) blot test
was positive for anti-Ro antibodies and anti-LA antibodies.
Slit lamp examination showed keratoconjunctivitis sicca.
This was supported by Schirmer’s test [Figure 1], showing
hypolacrimation with 7 mm wetting of the filter paper strip
in both eyes. Biopsy of the submandibular salivary gland and
lower lip [Figure 2] showed lymphocytic and macrophage
infiltration, chiefly around the salivary ducts. Thus, a rare
constellation of SS with distal renal tubular acidosis (dRTA)
and hypokalemic quadriparaesis was diagnosed.
Treatment with potassium chloride (diluted in intravenous
fluids) and oral potassium citrate solution was instituted
promptly since admission. Although hypokalemia and
quadriparaesis persisted for the first 3 days, there was
complete resolution of both these parameters thereafter. She
remains symptom-free on regular follow-up, but continues
to require potassium supplements.
Discussion
SS is a chronic autoimmune disease that may occur singularly
or along with other autoimmune diseases like systemic
lupus erythematosus, rheumatoid arthritis or systemic
sclerosis. Exocrine failure may affect the eyes, oral cavity,
skin or pancreas. The lungs, central and peripheral nervous
system, musculoskeletal apparatus and kidneys are other
organs to be affected. dRTA is a very rare feature of renal
involvement in SS, manifesting as failure of acidification
of urine with resultant alkaline urine and normal anion
gap metabolic acidosis.[1-3]
dRTA leads to sodium loss and
volume contraction, with compensatory raised aldosterone.
This causes increased resorption of sodium and increased
urinary loss of potassium (via upregulation and activation
of basolateral Na+
/K+
pumps) in the collecting duct of the
kidney and, hence, hypokalemia.[4]
Hypokalemia can be
severe enough to cause myopathy of varying degrees, from
minimal motor impairment to flaccid paralysis.
Hypokalemia can be associated with acid — base imbalance
(metabolic acidosis due to dRTA or metabolic alkalosis in
primary hyperaldosteronism) or can occur with normal
acid–base parameters (thyrotoxic periodic paralysis due to
increased intracellular movement of potassium, or familial
and sporadic periodic paralysis). A very useful parameter for
differentiation between renal and non-renal cause of HPP is
the trans-tubular potassium gradient (TTKG). It is calculated
as (urine potassium/plasma potassium) / (urine osmolality/
plasma osmolality). TTKG < 2 supports a non-renal cause
of hypokalemia while TTKG > 5 signifies increased renal
losses of potassium as seen in dRTA. This differentiation
has therapeutic implications as the potassium replacement
required in the non-renal cause of HPP is greater than in
renal HPP.[3]
The congregate of SS with renal involvement causing
dRTA and hypokalemia paralysis is extremely rare. When
investigating SS in HPP, it is imperative to look for historical,
Figure 1: Schirmer’s test showing hypolacrimation with 7 mm wetting
of the filter paper strip in both eyes (arrow)
Figure 2: Biopsy of the submandibular salivary gland and lower lip,
showing lymphocytic and macrophage infiltration, chiefly around the
salivary ducts (arrows)
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3. Diwan, et al.: An uncommon cause of quadriparaesis
496 Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4
histopathological, serological and immunological evidence.[2]
Also, the probable sequelae must be addressed while treating
the patient. These include sicca symptoms (dryness and
irritation of eyes, dysphagia, dental caries, hoarseness of
voice), musculoskeletal symptoms (fatigue, arthralgia),
nephrocalcinosis and chronic kidney disease and a greater
propensity to develop lymphoid malignancies (non-
Hodgkin’s lymphoma).[5,6]
Of special interest is SS in women
who become pregnant, as there is increased incidence of
neonatal lupus erythematosus with congenital heart block.[7]
Research in the treatment for SS has been unfruitful till now.
Thus, a diagnosis of SS may help in better understanding the
patient’s condition, but, unfortunately, it has little bearing
on treatment.
References
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Gupta RK, Al-Muzeiri IA. Sjögren’s syndrome presenting with
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Med 1998;18:167-70.
2. Khandelwal D, Bhattacharya S, Khadgawat R, Kaur S, Tandon
N, Ammini AC. Hypokalemic paralysis as a presenting
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2 cases. Indian Endocr Metab 2012;16:853-5.
3. Lin SH, LinYF, Halperin ML. Hypokalemia and paralysis. QJM
2001;94:133-9.
4. Wein AJ. Campbell-Walsh Urology Expert Consult. 10th
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5. Chen LH, Hsu PN, Chen MY, Lee KL, Hsieh SC, Yu CL. Renal
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6. Voulgarelis M, Skopouli FN. Clinical, immunologic, and
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7. Manthorpe R, Svensson A, Wirestrand LE. Late neonatal lupus
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How to cite this article: Diwan AG, Adukia SA, Annachhatre SV,
Chowdhury YS. Distal renal tubular acidosis and quadriparaesis in
Sjögren's syndrome: A cunning congregate. Med J DY Patil Univ
2014;7:494-6.
Source of Support: Nil. Conflict of Interest: None declared.
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