sodium-bicarbonate and Sjogren-s-Syndrome

Distal renal tubular acidosis is a relatively infrequent condition with complex pathophysiology that can present with life-threatening electrolyte abnormalities.. We describe a case of a 57-year-old Caucasian woman with previous episodes of hypokalemia, severe muscle weakness, and fatigue. Upon further questioning, symptoms of dry eye and dry mouth became evident. Initial evaluation revealed hyperchloremic metabolic acidosis, severe hypokalemia, persistent alkaline urine, and a positive urinary anion gap, suggestive of distal renal tubular acidosis. Additional laboratory workup and renal biopsy led to the diagnosis of primary Sjögren's syndrome with associated acute tubulointerstitial nephritis. After potassium and bicarbonate supplementation, immunomodulatory therapy with hydroxychloroquine, azathioprine, and prednisone was started. Nonetheless, her renal function failed to improve and remained steady with an estimated glomerular filtration rate of 42 ml/min/1.73 m. Cases of renal tubular acidosis should be carefully evaluated to prevent adverse complications, uncover a potentially treatable condition, and prevent the progression to chronic kidney disease. Repeated episodes of unexplained hypokalemia could be an important clue for diagnosis.

Topics: Acid-Base Equilibrium; Acidosis, Renal Tubular; Disease Progression; Female; Glomerular Filtration Rate; Humans; Hypokalemia; Immunomodulation; Middle Aged; Potassium; Sjogren's Syndrome; Sodium Bicarbonate; Trace Elements; Treatment Outcome

Renal tubular acidosis (RTA) is common in adults with primary Sjogren syndrome (pSS) but to date this condition has only been identified in 12 pediatric cases of pSS. Here we present the case of a 13-year-old, otherwise asymptomatic girl in whom the search for the etiology of incidentally found nephrocalcinosis led to diagnosis of distal RTA and nephrogenic diabetes insipidus secondary to SS-associated tubulointerstitial nephritis. Immunosupressive treatment and alkali/electrolyte supplementation resulted in stable renal function over the 6-year follow-up. A review of the literature focuses on two aspects of pSS: (1) the difficulties in diagnosing pSS in childhood and (2) clinical-pathological features, treatment and outcome of renal tubulointerstitial disease in childhood pSS. SS should be considered in older children, particularly females with otherwise unexplained RTA. A careful search for other renal dysfunctions is necessary, and renal biopsy may be of value in assessing the extent of renal damage and the need for immunomodulatory therapy.

Topics: Acidosis, Renal Tubular; Adolescent; Electrolytes; Female; Humans; Immunosuppressive Agents; Incidental Findings; Nephritis, Interstitial; Nephrocalcinosis; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

Primary Sjögren's syndrome (pSS) is a chronic slowly progressive autoimmune disease characterised by lymphocytic infiltration of salivary and lacrimal glands with varying degree of systemic involvement. Renal involvement, a recognised extraglandular manifestation of pSS, is commonly related to tubular dysfunction and generally manifests as distal renal tubular acidosis (RTA), proximal RTA, tubular proteinuria and nephrogenic diabetes insipidus. Untreated long-standing RTA is known to cause metabolic bone disease. Here, we present the report of a patient with sclerotic metabolic bone disease related to pSS with combined distal and proximal RTA and negative workup for other causes of sclerotic bone disease. A significant clinical and biochemical improvement, including recovery of proximal tubular dysfunction, was noted with alkali therapy. This case suggests the need to consider pSS in the diagnostic algorithm of a patient presenting with sclerotic bone disease.

Topics: Absorptiometry, Photon; Acidosis, Renal Tubular; Adult; Alkaline Phosphatase; Back Pain; Bone Density; Bone Diseases, Metabolic; Female; Humans; Potassium Citrate; Radionuclide Imaging; Sjogren's Syndrome; Skeleton; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Adult; Autoantibodies; Calcium; Calcium-Regulating Hormones and Agents; Diagnosis, Differential; Electromyography; Female; Humans; Hypokalemic Periodic Paralysis; Male; Muscle Weakness; Potassium; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

Topics: Acidosis, Renal Tubular; Adult; Cesarean Section; Female; Gestational Age; Heart Rate, Fetal; Humans; Nephrocalcinosis; Potassium Citrate; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Sjogren's Syndrome; Sodium Bicarbonate; Ultrasonography, Prenatal

A 61-year-old woman was admitted to our hospital because of muscle paralysis and was found to have severe hypokalemia. A gallium-67 scintigram revealed a positive accumulation in the bilateral salivary glands, and a labial minor salivary gland biopsy demonstrated a massive lymphocyte infiltrate around the salivary ducts. She was diagnosed with Sjögren's syndrome (SS) associated with renal tubular acidosis. Renal biopsy revealed tubulointerstitial nephritis with a mild focal infiltration of lymphocytes and plasma cells. These pathological features were compatible with SS with renal involvement. Acidosis and hypokalemia were corrected with sodium bicarbonate and potassium chloride, which relieved the patient's symptoms. Although steroid therapy has been reported to be effective in SS-associated tubulointerstitial nephritis, the patient's serum potassium level could be controlled without administering steroids during the first admission. Five years later, she was admitted again because of severe liver dysfunction attributed to autoimmune hepatitis. Oral administration of prednisolone resulted in the normalization of her transaminase levels, and the control of her serum potassium level became easier. It has been reported that patients with SS with salivary gland involvement tend to have hepatic complications, and those with hepatic complications tend to have renal involvement. Physicians should be aware of hepatic involvement, even if there is no liver dysfunction at the initial diagnosis of SS with salivary gland and renal involvement. It remains uncertain whether the administration of a low dose of steroids before the onset of autoimmune hepatitis might have prevented the development of liver dysfunction in our patient.

Topics: Acidosis, Renal Tubular; Administration, Oral; Female; Hepatitis, Autoimmune; Humans; Hypokalemia; Kidney; Lymphocytes; Middle Aged; Nephritis, Interstitial; Plasma Cells; Potassium Chloride; Prednisolone; Severity of Illness Index; Sjogren's Syndrome; Sodium Bicarbonate

A 62-year-old woman was diagnosed with severe osteomalacia caused by renal tubular acidosis associated with Sjögren's syndrome. She was treated with sodium bicarbonate, risedronate, alfacalcidol, and prednisolone (1 mg/kg). By 24 months, renal tubular acidosis was improved and the bone density had normalized. Here we report the successful amelioration of bone lesions through a multidisciplinary approach that improved renal tubular acidosis, with a special focus on treatment of the underlying inflammatory disorder with glucocorticoids.

Topics: Acidosis, Renal Tubular; Bone Density Conservation Agents; Drug Therapy, Combination; Etidronic Acid; Female; Glucocorticoids; Humans; Hydroxycholecalciferols; Middle Aged; Osteomalacia; Prednisolone; Risedronic Acid; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

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.

Topics: Acidosis, Renal Tubular; Administration, Intravenous; Adult; Antibodies, Antinuclear; Female; Glucocorticoids; Humans; Hypokalemic Periodic Paralysis; Potassium Chloride; Prednisolone; Quadriplegia; Salivary Glands; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

We present a 15-year-old girl with distal renal tubular acidosis (dRTA) appearing in what is probably a very early stage of primary Sjögren's syndrome. On the basis of tests evaluating renal handling of H+, we attempt to explain the mechanism of the urine acidification disorder. The inability to decrease urinary pH during systemic acidosis, together with the normal increase of urinary carbon dioxide partial pressure (pCO2) values after sodium bicarbonate and neutral phosphate loading, suggest a gradient-type dRTA. The inability to lower urinary pH in response to furosemide, accompanied by markedly increased urinary excretion of NH4, HCO3, Na, and K, points to a collecting tubule permeability disorder with bicarbonate leak to the tubular lumen. This patient had never been exposed to amphotericin B. To our knowledge, immune-related dRTA as a result of a gradient defect with bicarbonate leak into the tubular lumen has not been described.

Topics: Acidosis, Renal Tubular; Adolescent; Autoantibodies; Autoimmune Diseases; Bicarbonates; Carbon Dioxide; Diuretics; Female; Furosemide; Humans; Hydrogen-Ion Concentration; Kidney Tubules, Collecting; Permeability; Phosphates; Sjogren's Syndrome; Sodium Bicarbonate

The pathogenesis of renal potassium wasting and hypokalemia in classic renal tubular acidosis (type 1 RTA) remains uncertain. The prevailing theory is that K(+)-Na+ exchange is stimulated due to an inability of the distal tubule to establish a normal steep lumen-peritubular H+ gradient. We encountered a 42-year-old woman with type 1 RTA associated with Sjögren's syndrome, in whom renal potassium wasting and hypokalemia persisted despite sustained correction of systemic acidosis with alkali therapy and increased intake of potassium. In addition, plasma renin activity was markedly increased and the serum aldosterone level was upper-normal despite the hypokalemia. Increased intake of sodium resulted in suppression on the serum aldosterone and correction of renal potassium wasting and hypokalemia. This case shows that secondary hyperaldosteronism, possibly due to an impairment of sodium conservation in the distal tubule, may contribute to the loss of potassium from the distal tubule even after the correction of acidosis.

Topics: Acidosis, Renal Tubular; Adult; Aldosterone; Ammonium Chloride; Bicarbonates; Blood Gas Analysis; Female; Humans; Hydrogen-Ion Concentration; Hyperaldosteronism; Hypokalemia; Kidney; Potassium; Potassium, Dietary; Sjogren's Syndrome; Sodium; Sodium Bicarbonate; Sodium, Dietary; Urine; Water-Electrolyte Balance