Primary Sjogren's syndrome presenting as hypokalemic paralysis

Goroshi, Manjunath; Khare, Sanjay; Jamale, Tukaram; Shah, Nehal

doi:10.4103/0022-3859.194224

Cited by 11 publications

(8 citation statements)

References 12 publications

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“…Nonetheless, SS manifesting as hypokalemic paralysis or RTA is considered an enigma by most clinicians, as evidenced by the increasing number of case reports and small series (n < 10) describing this presentation. Case series with numbers greater than 10, exclusively reporting on clinical and laboratory features of SS‐RTA, are all from Asia 9–13 . These case reports and series contribute to the corpus of literature in the field and possibly outnumber the representation of RTA in cohorts of SS.…”

Section: Introductionmentioning

confidence: 99%

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Sandhya

2022

Int J of Rheum Dis

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Aim To describe the clinical and laboratory features of Sjögren's syndrome (SS) with renal tubular acidosis (RTA) from published literature. Methods A systematic search of indexed publications in all languages till December 2021 identified cases of SS with RTA (SS‐RTA) and were included if either antibody (anti‐SSA/anti‐SSB) or salivary gland histopathology were positive. Results There were 440 cases of SS‐RTA (63.9% from Asia, 95.5% women). The median (range) age was 37 (6‐78) years. Only 7.7% had a previous diagnosis of SS. Oral or ocular sicca symptoms were present in 63.7%. Positive ocular tests, oral tests, anti‐SSA, anti‐SSB and salivary gland histopathology were reported in 256/331 (77.3%), 123/128 (96%), 382/407 (93.9%), 298/379 (78.6%), and 246/268 (91.8%), respectively. Hypokalemic paralysis (HP) was the presenting feature in 63.6%; 25% had multiple episodes of HP and 8.4% had respiratory paralysis. Type 1, type 2, combined type 1 & 2, and type 4 RTA was seen in 388, 8, 38, and 3 patients, respectively. Proximal dysfunction and RTA complications were infrequently evaluated. Fanconi syndrome, nephrogenic diabetes insipidus, proteinuria, and low estimated glomerular filtration rate were found in 45, 21, 178, and 157, respectively. Nephrocalcinosis, renal stones, and osteomalacia were reported in 92, 79, and 72, respectively. Tubulointerstitial nephritis was found in 142 out of 152 renal biopsies. Conclusion SS‐RTA is an early manifestation of SS characterized by younger age and subclinical sicca symptoms. Although evaluated less frequently, oral sicca signs and salivary gland biopsy have a high positive yield. HP is the most common presentation. RTA is mostly distal; proximal dysfunction and complications were infrequently assessed.

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Section: Introductionmentioning

confidence: 99%

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Sandhya

2022

Int J of Rheum Dis

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“…5 Similar cases were reported by Goroshi et al where a case series of primary Sjogren's presenting as hypokalemic paralysis was made. 6 Objective evidence of lacrimal gland and minor salivary gland along with the serology supported the diagnosis of primary Sjogren's syndrome after excluding the evidence of cause for secondary Sjögren's syndrome. The patient had a very good response to replacement with potassium and bicarbonate with power improving to 5/5 in 24 hours of treatment.…”

Section: Discussionmentioning

confidence: 87%

A Case Report of Severe Hypokalemic Quadriparesis as the First Manifestation of Type 1 RTA in Primary Sjogren’s Syndrome

Sidharth¹

2021

JoARM

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Primary Sjogren’s syndrome is an autoimmune disorder primarily involving the exocrine glands but it can have a varied presentation. It can also present without classically described sicca symptoms. One-third of the patients experience extra-glandular involvement in the form of nonspecific arthralgia, myalgia, arthritis, parenchymal involvement of renal parenchyma, lung, and rarely nerves. Renal involvement in primary Sjogren’s can present with type 1 renal tubular acidosis (RTA) and is associated with a normal anion gap and hypokalemia. We, hereby, report a case of primary Sjogren’s syndrome presenting with type 1 RTA with hypokalemic quadriparesis as the first presenting feature.

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“…Hypokalemia is often the original presenting feature presumably due to antibody mediated impairment of potassium reabsorption [ 9 ]. Some cases are so severe that they present with flaccid paralysis related to the hypokalemia [ 13 ]. The hypokalemia is often self-limited while the acidosis persists and is later followed by nephrocalcinosis and nephrolithiasis as well as bone demineralization [ 26 – 28 ].…”

Section: Discussionmentioning

confidence: 99%

“…Rheumatologic conditions may not be initially considered in the differential if the presenting features are kidney stones or electrolyte abnormalities such as hypokalemia or metabolic acidosis [ 1 – 5 ]. However these can be the presenting manifestations of rheumatologic conditions that predominantly affect the renal tubules rather than the glomeruli [ 6 – 13 ].…”

Section: Introductionmentioning

confidence: 99%

Case report: disease mechanisms and medical management of calcium nephrolithiasis in rheumatologic diseases

et al. 2023

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Background Nephrolithiasis as a feature of rheumatologic diseases is under recognized. Understanding presenting features, diagnostic testing is crucial to proper management. Case presentation A 32 year old woman with a history of recurrent complicated nephrolithiasis presented to a rheumatologist for a several month history of fatigue, dry eyes, dry mouth, arthralgias. She had a positive double-stranded DNA, positive SSA and SSB antibodies. She was diagnosed with Systemic Lupus erythematosus (SLE) and Sjogren's syndrome and was started on mycophenalate mofetil. Of relevance was a visit to her local emergency room 4 years earlier with profound weakness with unexplained marked hypokalemia and a non-anion gap metabolic acidosis. Approximately one year after that episode she developed flank pain and nephrocalcinosis. She had multiple issues over the ensuing years with stones and infections on both sides. Interventions included extracorporeal shockwave lithotripsy as well as open lithotomy and eventual auto-transplantation of left kidney for recurrent ureteric stenosis. 24 h stone profile revealed marked hypocitraturia, normal urine calcium, normal urine oxalate and uric acid. She was treated with potassium citrate. Mycophenolate was eventually stopped due to recurrent urinary tract infections and she was started on Belimumab. Because of recurrent SLE flares, treatment was changed to Rituximab (every 6 months) with clinical and serologic improvement. Her kidney stone frequency gradually improved and no further interventions needed although she continued to require citrate repletion for hypocitraturia. Conclusions Nephrolithiasis can be a prominent and even presenting feature in Sjogrens syndrome as well as other rheumatologic diseases. Prompt recognition and understanding disease mechanisms is important for best therapeutic interventions for kidney stone prevention as well as treatment of underlying bone mineral disease.

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Primary Sjogren's syndrome presenting as hypokalemic paralysis

Cited by 11 publications

References 12 publications

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

A Case Report of Severe Hypokalemic Quadriparesis as the First Manifestation of Type 1 RTA in Primary Sjogren’s Syndrome

Case report: disease mechanisms and medical management of calcium nephrolithiasis in rheumatologic diseases

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