A case of Sjögren’s syndrome presenting as hypokalemic myopathy due to distal renal tubular acidosis

We report a case of hypokalemic myopathy with distal renal tubular acidosis which leads to the diagnosis of Sjögren’s syndrome (SS). A 67-year-old man was admitted to our department for progressive muscle weakness and myalgia. Laboratory data demonstrated hypokalemia with hyperchloremic acidosis, elevated muscle enzyme and inability to acidify urine. Findings of interstitial nephritis, which is consistent with renal tubular acidosis in SS, were found on renal biopsy. Remarkable changes of the renal tubule, including tubular atrophy, dilatation and intratubular casts, were observed. Potassium replacement therapy normalized the marked elevated urinaryβ ₂-microglobulin excretion as well as the improvement of muscle symptoms. Taken together with our findings, it it suggested that sustained hypokalemia may partly contribute to the exacerbation of the pre-existing renal tubular disorder in SS.     

A primary Sjögren’s syndrome patient with distal renal tubular acidosis, who presented with symptoms of hypokalemic periodic paralysis

Although renal tubular acidosis (RTA), secondary to autoimmune interstitial nephritis, develops in a large proportion of patients with Sjögrens syndrome (SS), most of the subjects are asymptomatic. Here, we shall present a 39-year-old female patient who came to us with hypokalemic periodic paralysis (HPP), and who was later diagnosed with distal RTA. The patient, who had xerostomia and xerophthalmia for a long period of time, was diagnosed with primary SS from serologic and histologic findings. The patient recovered by being prescribed potassium replacement therapy. Although renal biopsy was not performed, corticosteroids were administered because HPP indicated severe interstitial nephritis. HPP did not reoccur during a 2-year follow-up period. We also review cases with SS-related distal RTA and HPP.     

Osteomalacia revealing primary Sjögren’s syndrome in a Tunisian woman with distal renal tubular acidosis

Introduction

Sjögren’s syndrome (SS) is a chronic disorder affecting primarily exocrine glands associated with lymphocytic infiltrates. The most common renal disease in SS is tubulo-interstitial nephritis, responsible for renal tubular acidosis (RTA) in 20% of the patients. It is often latent and can be rarely complicated by osteomalacia.

The spectrum of renal tubular acidosis in paediatric Sjögren syndrome

OBJECTIVES: Renal tubular acidosis (RTA) is a well-recognized extraglandular complication of adult Sj?gren syndrome (SS) but has been reported only rarely in paediatric SS. We wished to describe the natural history of RTA in paediatric SS. METHODS: We performed a chart and literature review. Inclusion criteria were primary or secondary SS with onset before 18 yr of age, complicated by RTA before 18 yr of age. RESULTS: Twelve cases were identified: two from chart review and 10 from the literature. RTA was mostly associated with primary SS. RTA was detected at the onset of SS or up to 9 yr later. The clinical spectrum ranged from nearly silent to life-threatening, with plasma pH and serum potassium as low as 7.0 and 1.2 mEq/l, respectively. Hypokalaemia was present in 92%. Half the patients presented with profound weakness or paralysis, most likely from hypokalaemia. Proximal, distal and mixed RTA were detected, reflecting a diffuse 'tubulopathy' from interstitial nephritis, which was the predominant histopathological finding. Diabetes insipidus was the most frequent renal comorbidity. The RTA stabilized in 82% of the cases and resolved in one case. Only one patient had long-term unstable RTA. CONCLUSIONS: RTA is an under-recognized complication of paediatric SS. It can be life-threatening in the acute phase but generally has a good long-term renal outcome. SS should be considered in the older child with otherwise unexplained RTA. Likewise, RTA should be excluded in children and adolescents with SS who develop weakness, fatigue or growth failure. Early recognition would reduce long-term complications such as growth failure.     

Successful treatment of osteomalacia caused by renal tubular acidosis associated with Sjögren’s syndrome

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.     

A case of Sjögren's syndrome complicated by nephrogenic diabetes insipidus and renal tubular acidosis

We describe the case of a 46-year-old woman with Sjögren's syndrome (SS) presenting with a 6-year history of polyuria and polydipsia. Laboratory data revealed hyperchloremic metabolic acidosis, a normal anion gap, and an inability to acidify urine following an acid loading test and to concentrate the urine in response to water deprivation and antidiuretic hormone administration. Lymphocyte infiltration in the interstitium was found on renal biopsy. These findings allowed us to diagnose distal renal tubular acidosis (RTA) and nephrogenic diabetes insipidus (NDI). Steroid pulse therapy resulted in normalization of the blood pH, but failed to remit the inability to concentrate the urine. These observations suggest therapeutic applications for RTA in SS, and that further investigation is required to design a therapeutic strategy for NDI in SS.     

A case of interstitial cystitis accompanying Sjögren’s syndrome

A 51-year-old woman with interstitial cystitis was referred for screening of autoimmune disease. She was positive for results of Schirmer and Saxon tests and for ss/A and ss/B antibodies. Autoantibodies to type 3 muscarinic acetylcholine receptor were detected in the patients serum by Western blotting. We diagnosed her as having interstitial cystitis accompanying Sjögrens syndrome. To enable early treatment, interstitial cystitis should be considered when patients with Sjögrens syndrome complain of urinary symptoms.     

Hypokalemic periodic paralysis in Sjogren’s syndrome secondary to distal renal tubular acidosis

We report a 53-year-old Turkish female presented with progressive weakness and mild dyspnea. Laboratory results demonstrated severe hypokalemia with hyperchloremic metabolic acidosis. The urinary anion gap was positive in the presence of acidemia, thus she was diagnosed with hypokalemic paralysis from a severe distal renal tubular acidosis (RTA). Immunologic work-up showed a strongly positive ANA of 1:3,200 and positive antibodies to SSA and SSB. Schirmer’s test was abnormal. Autoimmune and other tests revealed Sjögren syndrome as the underlying cause of the distal renal tubular acidosis. Renal involvement in Sjogren’s syndrome (SS) is not uncommon and may precede sicca complaints. The pathology in most cases is a tubulointerstitial nephritis causing among other things, distal RTA, and, rarely, hypokalemic paralysis. Treatment consists of potassium repletion, alkali therapy, and corticosteroids. Primary SS could be a differential in women with acute weakness and hypokalemia.     

A case of primary Sjögren’s syndrome presenting primarily with central nervous system vasculitic involvement

Sjögren’s syndrome is primarily a chronic systemic autoimmune disease that affects exocrine organs. Neurologic symptoms frequently present as peripheral neuropathy due to small vessel vasculitis. Type and prevalence of central nervous system involvement are still controversial. In this report, we present a 35-year-old woman with primary Sjögren’s syndrome with central nervous system vasculitic involvement.     

A case of primary Sjögren’s syndrome with pulmonary-limited Wegener’s granulomatosis

A 60-year-old woman had a history of dyspnea for 5–6 weeks. The chest radiograph and computed tomography scans revealed bilateral patchy reticulonodular pattern. The patient had positive test results for antineutrophil cytoplasmic antibody against proteinase-3 (c-ANCA), antinuclear antibody and anti-Ro antibody. According to European Study Group on Classification Criteria for Sjögren’s Syndrome, the patient was diagnosed as primary Sjögren’s syndrome based on the presence of clinical features, positive findings on Schirmer’s test and parotis scintigraphy. Lung biopsy obtained by wedge resection showed granulomatous inflammation with extensive multinuclear giant cells involving the lung parenchyma and vascular structures. There was neither upper airway nor renal involvement. Thus, the patient was simultaneously diagnosed as pulmonary-limited Wegener’s granulomatosis. With this unique case, we would like to emphasize that the awareness of ANCA-associated vasculitis as a diagnostic possibility in primary Sjögren’s syndrome is important during the work-up of lung lesions.     

Renal tubular acidosis, Sjögren syndrome, and bone disease

BACKGROUND: There has been disagreement about whether osteomalacia (adult rickets) occurs in adults with type 1 (distal) renal tubular acidosis (RTA1). Therefore, after finding scapular pseudofractures in a patient with RTA1 and Sj?gren syndrome, we decided to survey other patients with RTA to learn whether osteomalacia occurred in others and, if it did, whether it was necessarily associated with the presence of Sj?gren syndrome. METHODS: We examined the hospital records and laboratory findings of 250 patients with codes for RTA, 124 with codes for osteomalacia, and 20 with codes for Sj?gren syndrome who were seen at a university-affiliated acute care municipal hospital since 1990. Further detailed survey was then limited to patients older than 15 years and excluded those with potentially confounding causes of bone disease such as chronic renal insufficiency or sickle cell disease. Seven adults with RTA1 were thereby identified. RESULTS: Two adults with RTA1 had radiological and biochemical findings compatible with osteomalacia, and 1 had findings compatible with Sj?gren syndrome. A third patient without Sj?gren syndrome had biochemical findings suggestive of osteomalacia. CONCLUSIONS: Osteomalacia seems to occur in some adult patients with RTA1, and not only in association with Sj?gren syndrome. We found no biochemical evidence of osteomalacia in the patients with Sj?gren syndrome who did not have RTA.     

Nephrocalcinosis: a rare presenting manifestation of primary Sjögren’s syndrome

Abstract

Renal involvement in primary Sjögren’s syndrome (pSS) is not uncommon. Autoimmune tubulointerstitial disorders and distal renal tubular acidosis (dRTA) account for majority of the cases of renal involvement. While dRTA may precede the onset of sicca syndrome in pSS, nephrocalcinosis as a presenting manifestation of pSS is rare. Here, to emphasize the need for initiating investigations for pSS in any patient presenting with nephrocalcinosis due to dRTA, we report a 21-year-old woman presenting with nephrocalcinosis long before pSS was objectively diagnosed.     

Nephrocalcinosis: a rare presenting manifestation of primary Sjögren’s syndrome

Renal involvement in primary Sjögren’s syndrome (pSS) is not uncommon. Autoimmune tubulointerstitial disorders and distal renal tubular acidosis (dRTA) account for majority of the cases of renal involvement. While dRTA may precede the onset of sicca syndrome in pSS, nephrocalcinosis as a presenting manifestation of pSS is rare. Here, to emphasize the need for initiating investigations for pSS in any patient presenting with nephrocalcinosis due to dRTA, we report a 21-year-old woman presenting with nephrocalcinosis long before pSS was objectively diagnosed.     

Unusual presentation of triple A syndrome mimicking Sjögren’s syndrome

Triple A syndrome is a rare autosomal recessive disorder characterized by alacrima, achalasia, and adrenal insufficiency. Sjögren’s syndrome (SS) is a chronic inflammatory disorder manifested primarily by diminished lacrimal and salivary gland secretions, resulting in symptoms of dry eyes and dry mouth, the so-called “sicca complex”. However, a variety of other manifestations also can occur, which can be termed “nonexocrine manifestations”. One of the frequent nonexocrine manifestations is dysphagia. In this paper, we present an unusual form of Triple A disease mimicking Sjögren’s syndrome, which leads to a challenging diagnosis.     

Hypokalemic paralysis and osteomalacia secondary to renal tubular acidosis in a case with primary Sjögren's syndrome

A 39-year-old Japanese woman was admitted to our hospital for severe weakness owing to potassium deficiency caused by type 1 renal tubular acidosis (RTA1). Sicca complex, serological tests, and lip biopsy revealed that she had Sjögren's syndrome (SS). Acidosis was corrected by alkali supplement treatment. She also had an impaired renal function with proteinuria, and high absorbance on Ga scintigram was recognized in both kidneys. She was taking warfarin potassium after aortic valve substitution due to aortic regurgitation, therefore renal biopsy was not performed. Prednisone (20?mg/day) was administered for renal inflammation. One month later, she suffered severe chest wall pains with some local tender points over the costae of both sides, which was presumed to be due to pseudofractures based on osteomalacia. Hypokalemic paralysis and osteomalacia should be taken into consideration in the diagnosis of SS with RTA1.     

A case of anti-neutrophil cytoplasmic antibody-associated vasculitis masquerading as Sjögren syndrome

Anti-neutrophil cytoplasmic antibody (ANCA) -positive vasculitis is a small-vessel vasculitis that affects multiple body systems. Salivary gland involvement in ANCA-associated vasculitis is rare. When present, it mimics infection or malignancy, which might lead to misdiagnosis. In this report, we describe a 72-year-old man who presented with parotid and submandibular gland pain and swelling in addition to dry mouth and eyes. He had bilateral non-tender parotid gland lumps and no lymphadenopathies. Laboratory tests were positive for ANCA, hematuria, and proteinuria but negative for Anti-Ro and -La. He was treated with corticosteroids and cyclophosphamide for acute kidney injury. Unfortunately, the patient died a few months later. This case report sheds light on a rare manifestation of salivary gland involvement in ANCA-associated vasculitis that mimics Sjögren syndrome and the challenges associated with its diagnosis and treatment.     

Hodgkin lymphoma as a complication of primary Sjögren’s syndrome

Sjögren’s syndrome (SS) is a chronic autoimmune disease that is characterized by lymphocytic infiltration of the exocrine glands, mainly the salivary and lachrymal glands, usually manifesting with xerostomia and xerophthalmia. Around 50% of patients with primary SS develop systemic complications, lymphoma being the most feared of these. The majority of these neoplasias originate from B cells and are of the non-Hodgkin type. We describe here a rare case of SS in which the patient developed a Hodgkin lymphoma. We also review the literature on this subject.     

Sjögren’s syndrome in childhood

This review presents our 10-year experience with children diagnosed with Sjögren’s syndrome (SS). Patients between the ages of 9 and 17 years had abnormalities in laboratory values consistent with but not entirely diagnostic of those required to diagnose SS in adults. The spectrum of clinical manifestations suggests that the SS clinical phenotype in children is more variable than that in adults. Here, we review manifestations of SS in children. Our patients were treated with hydroxychloroquine, despite the lack of prospective data about effects on SS progression and/or autoantibody spreading. Patients have been followed for between 3 and 6 years without substantial progression of their disease or change in autoantibody status. Longer term follow-up (10–20 years) is needed to define the natural history of SS in childhood and its treatment outcomes. Prospective validation of SS criteria in childhood could facilitate assessment of the utility of hydroxychloroquine and other therapies.     

Hodgkin lymphoma as a complication of primary Sjögren’s syndrome

Abstract

Sjögren’s syndrome (SS) is a chronic autoimmune disease that is characterized by lymphocytic infiltration of the exocrine glands, mainly the salivary and lachrymal glands, usually manifesting with xerostomia and xerophthalmia. Around 50% of patients with primary SS develop systemic complications, lymphoma being the most feared of these. The majority of these neoplasias originate from B cells and are of the non-Hodgkin type. We describe here a rare case of SS in which the patient developed a Hodgkin lymphoma. We also review the literature on this subject.