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British Journal of Healthcare and Medical Research - Vol. 11, No. 3
Publication Date: June 25, 2024
DOI:10.14738/bjhmr.113.17073.
John, D., Sebastian, R., Elias, G., Fabian, G., Karla, A., & Alejandro, R. (2024). Case Report: Renal Tubular Acidosis as The Initial
Presentation of Sjogren's Syndrome. British Journal of Healthcare and Medical Research, Vol - 11(3). 291-296.
Services for Science and Education – United Kingdom
Case Report: Renal Tubular Acidosis as The Initial Presentation
of Sjogren's Syndrome
Dorado John
Mexican Social Security Institute: General Hospital of Zone number 50;
Autonomous University of San Luis Potosí – Mexico
Ramirez Sebastian
Mexican Social Security Institute: General Hospital of Zone number 50;
Autonomous University of San Luis Potosí – Mexico
Galica Elias
Mexican Social Security Institute: General Hospital of Zone number 50;
Autonomous University of San Luis Potosí – Mexico
Garza Fabian
Mexican Social Security Institute: General Hospital of Zone number 50;
Autonomous University of San Luis Potosí – Mexico
Aguirre Karla
Mexican Social Security Institute: General Hospital of Zone number 50;
Autonomous University of San Luis Potosí – Mexico
Rico Alejandro
Mexican Social Security Institute: General Hospital of Zone number 50;
Autonomous University of San Luis Potosí – Mexico
ABSTRACT
Sjögren's syndrome (SSjP) is a chronic multisystem autoimmune disorder
characterized by inflammation of the lacrimal and salivary glands, with consequent
dryness of the eyes and mouth and occasional glandular enlargement, it occurs in a
primary form, not associated with other autoimmune rheumatic diseases. We
present the case of a 36-year-old female patient with Sjögren's syndrome whose
initial clinical manifestation was extraglandular, with the kidney as the main organ
affected in the form of chronic hypokalemia and distal renal tubular acidosis, which
led to immunological markers being performed. for Sjögren's syndrome, the
diagnosis of this autoimmune exocrinopathy allowed, achieving clinical remission
and stabilization of renal function with the use of steroids.
Keywords: Sjögren's syndrome, autoimmune exocrinopathy renal tubular acidosis,
hypokalemia, tubulointerstitial nephri.
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British Journal of Healthcare and Medical Research (BJHMR) Vol 11, Issue 03, June-2024
Services for Science and Education – United Kingdom
INTRODUCTION
Primary Sjögren's syndrome (SSjP) is defined as an autoimmune disorder of the connective
tissue with the presence of B lymphocyte infiltration in the epithelial tissues of the glands. B
lymphocytes are also found in extraglandular epithelia such as the lungs, gastrointestinal tract,
hepatobiliary tract, and renal epithelium.(1).
Classic clinical manifestations of SSjP (primary Sjögren's syndrome) include dryness, defined
as dry eyes and mouth, which occurs in more than 90% of patients (2). Dry mouth causes pain
when swallowing and tooth decay; Regarding dry eyes, it manifests itself as
keratoconjunctivitis sicca that presents the following symptoms: eye pain, irritation and
sensation of a foreign body in the eyes.
On the other hand, extraglandular manifestations of SSjP are found in variable forms and can
affect most organic systems. Some of the known presentations include tracheobronchial sicca
and interstitial pneumonia in the respiratory system, and pericarditis and pulmonary
hypertension in the cardiovascular system. Other signs and symptoms may include dysphagia,
arthralgia, myalgia, hypothyroidism, and skin manifestations, including nonblanching skin
rashes (3). Since the condition is caused by the proliferation of B lymphocytes, the development
of lymphoproliferative disorders is a major concern.
SSjP can also be secondary to other autoimmune conditions, including but not limited to
systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and systemic sclerosis
(scleroderma) (1). The prevalence of renal involvement in SSjP has been reported to range from
approximately 10% to 30%.(4).
Kidney disease in SSjP is due to 2 different pathophysiological processes: (1) Epithelial disease
with significant lymphocytic infiltration, resulting in different conditions such as
tubulointerstitial nephritis (TIN), electrolyte alterations such as hypokalemia, distal renal
tubular acidosis, proximal renal tubular acidosis , Fanconi syndrome, diabetes insipidus,
Gitelman syndrome, nephrolithiasis and nephrocalcinosis, and (2) non-epithelial disease that
can lead to glomerulopathy as a result of an immune complex-mediated process (5). (1)
Epithelial disease with significant lymphocytic infiltration, resulting in different conditions
such as tubulointerstitial nephritis (TIN), electrolyte alterations such as hypokalemia, distal
renal tubular acidosis, proximal renal tubular acidosis, Fanconi syndrome, diabetes insipidus,
Gitelman syndrome, nephrolithiasis and nephrocalcinosis, and (2) non-epithelial disease that
can lead to glomerulopathy as a result of an immune complex-mediated process
CASE REPORT
A 36-year-old female patient was admitted to the nephrology service due to recurrent
symptomatic severe hypokalemia, accompanied by normal anion gap metabolic acidosis and
elevated creatinine. She reported marijuana abuse and toluene inhalation in her youth. She has
multiple tattoos. She was hospitalized in 2021 due to weakness associated with hypokalemia,
hypophosphatemia, hyperchloremia, since then with elevated creatinine, she received
intravenous electrolytes with improvement and was discharged.
He was admitted with blood pressure 90/60 mmHg, with the following findings: P 1.5, Cal 8.6,
Cl- 131, K+ 2.77, Na+ 145, Mg 2.24, Cr 1.90, Hb 9.9 HTO 33.3, albumin 3.34 g/dl, venous blood
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John, D., Sebastian, R., Elias, G., Fabian, G., Karla, A., & Alejandro, R. (2024). Case Report: Renal Tubular Acidosis as The Initial Presentation of
Sjogren's Syndrome. British Journal of Healthcare and Medical Research, Vol - 11(3). 291-296.
URL: http://dx.doi.org/10.14738/bjhmr.113.17073.
gases: ph 7.23, pco2 15, hco3 8.4, anion gap 8.3, ego density 1.01, ph 6.73, proteins 25, nitrites
-, leukos 5-10/field, urinary sediment with the presence of some granular casts and leukocyte
casts. Proteinuria/creatinuria ratio 656.7 mg/g, urinary electrolytes Volume 9,140 ml, CLU 243,
KU 116.9, NAU 319.9 mEq/24h, citrate 197 mg/24h, urinary anion gap +193.8 MEQ/L. Renal
ultrasound RD 91*53*50, RI 87*46*43 both lobulated, hyperechoic cortex, decreased
vascularity. Positive serologies for ANAs 1:160 +++ 1:1280 +, ANTI-SSA RO IgG 187, ANTI-SSB
LA IgG 93, negative ANTI-DNA-DS and complement levels were normal.
An ultrasound-guided renal biopsy was performed, reporting 29 glomeruli with data of chronic
hypoperfusion, diffuse cicatricial global and segmental sclerosis, active and chronic
tubulointerscitial nephritis, focal acute tubular injury, grade II interstical fibrosis 40-50% mild
arteriolosclerosis and chronic arteriolopathy. (Illustration 1). Immunofluorescence was
negative (Table 1).
Sjögren's syndrome was concluded with chronic tubulointerstitial nephritis type kidney
disease manifested with distal renal tubular acidosis type 1 (illustration 2). The patient
received steroids and rituximab as well as a potassium citrate supplement. She presented a
decrease in creatinine to 1.31 mg/dl, with normokalemia but persistent metabolic acidosis.
Illustration 1: Renal Biopsy Active and Chronic Tubulo-Interstitial Nephritis. O Acute Focal
Tubular Lesion with Moderate Regenerative Changes of The Epithelium. O Chronic Glomerular
Hypoperfusion With Diffuse Global and Segmental Sclerosis. O Interstitial Fibrosis Grade Ii (40-
50%). Or Mild Arteriolosclerosis. O Chronic Arteriopathy
Table 1: Direct Immunofluorescence Study in Dewaxed Tissue
Inmunoreact Result
IgG Positive with pattern linear in the membranes
IgA Negative
IgM Negative
C1q Negative