WIMJOURNAL, Volume No. 3, Issue No.

1, 2016 pISSN 2349-2910

eISSN 2395-0684

CASE REPORT
A case of idiopathic hypoparathyroidism with Systemic Lupus Erythematosus
Nillawar AN1, Mitul Chhatriwala2. #
Associate Professor, Department of Biochemistry,S.B.K.S.Medical Institute & Research
Centre, Vadodara, Gujarat1, Assistant Professor, Department of Biochemistry,
Pramukhswami Medical College, Karamsad, Gujarat2
# Both the authors contributed equally

Abstract: generalised weakness. She is a known case

Hypoparathyroidism is a rare disease. hypothyroidism and systemic lupus
The main cause of hypoparathyroidism is erythematous for 15 years. Past history of
postsurgical hypoparathyroidism. However, repeated pericardial effusion. On admission,
cases of hypoparathyroidism in patients radiological findings on MRI (plain &
suffering from SLE exist although it is contrast) were multiple lacunar infarcts in
uncommon. We present the case of a woman basal ganglia on both side and calcification in
suffering both from systemic lupus caudate nucleus, putamen, pulviener, internal
erythematosus and hypoparathyroidism. This capsule. Laboratory findings were total serum
reported association of hypoparathyroidism calcium 4.5mg/dl, PTH 2.02pg/ml, ionized
with lupus expands the spectrum of endocrine calcium 0.67mg/dL, 25-hydroxy vitamin-D
disorders seen in this disease. We suggest that 70ng/ml. Hypercaliuria present. ANA;
there may be a common underlying positive, Anti-ds DNA antibodies positive. All
pathophysiological process linking these other laboratory findings are within normal
diseases. limit. This association of hypoparathyroidism
with SLE is a rare association.
Case report:
We present a case of 50-year-old Keywords: Hypoparathyroidism, SLE,
female resident of Madhya Pradesh, India who Hypercalciuria, CaSR, Basal ganglion
had chief complaint of seizures and calcification, Hypothyroidism,

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Introduction:

Hypoparathyroidism is rare disease. Broadly report a case of PTH deficient

speaking, causes of hypoparathyroidism can hypoparathyroidism associated with
be classified as PTH- deficient and PTH- hypothyroidism and SLE.
sufficient (Psuedo hypoparathyroidism type
Case Report:
1 and type 2). Causes of PTH-deficient
Hypoparathyroidism include post thyroid 50-year-old female, presents with
surgery, infiltration of parathyroid glands, complaints of cough with yellow
neck radiation which impairs either PTH expectoration for 15 days. She has pedal
synthesis or secretion.(1)This can be caused oedema and generalised weakness and
by autoantibodies to parathyroid gland and arthralgia on and off for 15 years. She has
related molecules.(2)Laboratory values of seizure disorder for 15 years and is treated
intact PTH, Serum Ionized calcium, 25 (OH) with anti-seizure treatment. Additionally she
D levels, Serum Phosphate, Glomerular has documented severe hypocalcaemia for
filtration rate (GFR), serum Magnesium and 10 years (Range of hypocalcaemia is in
evaluation for relative hypercalciuria help to between Total calcium=4 to 5 mg %). On
dissect out causes of Hypocalcaemia. examination, patient does not have any sign
Screening for autoimmune polyglandular suggestive of neuromuscular irritability. Her
syndrome helps to understand syndromal laboratory values from recent encounter are
presentation of hypocalcaemia.(3)Here we presented in Table:

Haematological parameters:

Test Value Remark

Hb 11.6 Gm% Slightly anaemic
MCV, MCH, MCHC, 97fl/31.8/32/10220/3.2 lakhs Within Normal Limit
TLC, DLC, Platelets
ESR 76mm/hr Raised

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Serum Chemistry:

Test Value Normal range Comment

Sr. ALT 24 IU/L 4-10 IU/L
Sr. AST 20 IU/L 4-14 IU/L
Sr. ALP 90 IU/L 40-120 IU/L
Sr. Total Protein: 8gm% 7.0-7.5 mg/dl
Sr. Albumin 4.1gm% 3.5-5 mg/dl
Sr. Urea 21 mg/dl 15-40 mg/L
Sr. Creatinine 1.1mg/dl 0.9-1.1 mg/L
Sr. Na 138 mEq/L 136-145 mEq/L
Serum K 4.0 mEq/L 3.5-5.5 mEq/L
Serum Cl 102 meq/L 95-105 mEq/L
Serum Total Calcium 4.7 mg/dl 9-11 mg/dl
Serum Ionized 0.67 mg/dl 4.5-5.5 mg/dl Severe Hypocalcaemia
Calcium
Serum Phosphorus 4.8 mg/dl 3.5-5.5 mg/dl
Vit D (OH,25) 70 ng/ml 20-100 ng/ml Sufficient status
Serum Mg 1.9 mg/L 1.8-2.0 mg/dl Rules out Hypomagnesaemia
Serum PTH 2.02 pg/ml 10-65pg/ml
Serum TSH 10.4 IU/L 0.4-4.0 IU/L Diagnostic of
Hypothyroidism
*Urinary Calcium 384 mg/24 hr 250-300 mg/24 hr Relative Hypercalciuria
Urinary Ca/Cr 0.2 Less than 0.14 Indicates Hypercalciuria
eGFR 120 ml/min/1.73m2 110-120 ml/min Rules Out Kidney failure
Sr ANA Level Positive in 1:160 titre Sensitive for SLE, These
much high titres s/o SLE
Anti ds-DNA Ab Positive Highly specific for SLE

*Hypercalciuria is defined as urinary excretion of more than 250 mg of calcium per day in women or
more than 275-300 mg of calcium per day in men while on a regular unrestricted diet.

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eISSN 2395-0684

MRI brain (plain & is the direct first messenger for these
contrast) showed multiple lacunar infarct in receptors.(6) Activating mutations of CaSR
basal ganglia bilaterally, calcification in results in suppressed secretion of PTH. This
caudate nucleus, putamen, pulvinar, internal results in hypercalciuria as activation in
capsule (Figure y). On Chest X-ray, pleural kidney tubular cells result in suppression of
and pericardial effusion is seen.(4,5) paracellular reabsorption of Ca and Mg ions
resulting in hypocalcaemia and
Family history and
hypomagnesemia.(5,4)
pedigree analysis was inconclusive in this
patient. Antibodies to CASR are shown to be
responsible for hypoparathyroidism in
Discussion:
autoimmune polyendocrine syndrome type 1
This patient has hypoparathyroidism in one reported study.(7)Usually APS type 1
with deficient parathormone. There was no presents in childhood to adolescence.
history of removal of parathyroid or surgical Addisons disease and type 1 diabetes are the
manipulation of thyroid which rules out associates in this syndrome.
inadvertent damage to parathyroid glands. In
Acquired hypoparathyroidism patients
cases without such iatrogenic removal, age of
have antibodies reactive exclusive to CaSR as
presentation and accompanying illness can
observed by the experiment done by Yangxin
suggest the diagnosis.(3) Early age of
Li and et al which showed the presence of
presentation is associated with some genetic
such antibodies. In this study, 8 female
diseases like DiGeorge syndrome or HDR
Patients were having associate
syndrome, a group of hypoparathyroidism (8)
hypothyroidism.
associated with hypomagnesemia. In familial
isolated hypoparathyroidism, there is Prevalence of hypothyroidism and
activating mutation of CaSR. CaSR is presence of anti-thyroid antibodies (Anti-TPO,
expressed extracellularly on parathyroid gland anti-thyroglobulin) is quite high in SLE.(9,10)
and renal distal tubules. Free ionized calcium

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Known causes of Hypoparathyroidism due to Impaired PTH:

Known Syndrome or Onset Presentation and Mechanism

diagnosis association with
H/o Surgical removal of Hypocalcaemia Damage to
thyroid or parathyroid/ persistent after 6 parathyroid glands
radiation/cancer months of surgery
metastasis/hemochromatosis
DiGeorge syndrome Early onset, facial Branchial arch
dysmorphology, dysmorphogenesis
cardiac anomalies
HDR syndrome Early Onset Renal disease,
sensorineural
deafness
Familial Hypoparathroidism Neonatal/Infantile Associated with Genetic diseases
with Hypomagnesemia presentation hypomagnesemia. with Activating
Some are associated CaSR mutations/
with Bartters defective
syndrome Parathormone
Family history. secretion
Can be autosomal
recessive or
dominant
Autoimmune polyendocrine Late onset/ Addisons disease. Mutations in
Neoplasis(APN) type 1 Or Post partum. Type 1 Diabetes autoimmune
Hypothyroidism, regulator region.
Waxing and waning Few cases are seen
of disease. with autoantibodies
against CaSR
Acquired Late Onset In one of the study
Hypoparathyroidism strongly associated Antibodies against
withhypothyroidism CaSR receptors
and female gender

In this patient, SLE is diagnosed by Seizure disorder and positive anti ds-DNA
(11)
criterion laid down by American College of and ANA. She has hypothyroidism and
Rheumatology as patient has evidence of hypoparathyroidism. She is a severe and
pericardial effusion, arthralgia, history of long-standing case of hypocalcaemia with

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symmetrical basal ganglion calcification and assay of anti CaSR antibody or mutation
long history of seizure disorder (MRI testing for the same.
Images attached). Similar pattern of
Presence of hypoparathyroid
calcification has been reported (2,11,1517)
(12-14)
dysfunction in SLE is very rare
previously. Despite severe deficiency
though evidence of anti CaSR antibodies in
of ionized calcium, patient did not show any
SLE is not found in literature. We suggest
signs of neuromuscular irritability or any
that there may be a common underlying
ECG changes. She has signs of cardiac
pathophysiological process linking these
failure in terms of pedal oedema and signs
diseases which could be genetic or even
of pericardial effusion. (Most likely disease
autoimmunity.
process of SLE). She has laboratory
evidence of hypothyroidism in terms of So this is a case of late onset, female
raised TSH. In spite of very low calcium, of SLE with hypothyroidism and PTH-
kidneys are excreting large amount of deficient-hypoparathyroidism with severe
calcium suggest activation of CaSR hypocalcaemia and hypercalciuria and
receptors. (See table). We did not perform normomagnesemia.

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Author for Correspondence:

Dr.Nillawar AN.
Associate Professor,
Department of Biochemistry, S.B.K.S.Medical Institute & Research Centre,
Vadodara, Gujarat, India
Email:nilawaranup@gmail.com

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