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Informative Article | Medicine | India | Volume 13 Issue 6, June 2024 | Rating: 5.5 / 10
Sjogren's Syndrome Presenting as Hypokalemic Periodic Paralysis
Dr. Manjiri Naik [5] | Dr. Siddhraj Vinod Paramshetti [2] | Dr. Sushen Ghadge [2] | Dr. Shubham Patel [2] | Dr. Kartik Doshi | Dr. Abhay Bhosale [2] | Dr. Kahaan Shah [2]
Abstract: Sjogren's syndrome is a prototype autoimmune disease characterised by lymphocytic infiltration of the exocrine glands resulting in xerostomia, dry eyes, and profound B-cell hyperactivity. The syndrome has unique features since it presents with a wide clinical spectrum from organ-specific to systemic disease; can occur alone or in association with other systemic rheumatic diseases. Distal renal tubular acidosis is a known cause of hypokalemia, which may rarely be severe enough to present as hypokalemic paralysis. Sjogren's syndrome presenting for the first time with hypokalemic paralysis due to dRTA in a patient with no sicca symptoms is even rarer. The only evidence of underlying Sjogren's syndrome in our patient was positive serology and evidence of RTA. Case report:36 year, Female not a known case of any comorbidities presented with sudden onset proximal muscles weakness of all four limbs in one day. No history of fever, diarrhoea, steroid or diuretic use, animal bite or dry eyes or joint pain. There was no sensory or autonomic involvement. On examination she was vitally stable with areflexic quadriparesis with no cranial nerves involved. Lab wise potassium was 2.6 with arterial blood gas analysis suggestive of metabolic acidosis and trans-tubular potassium gradient of suggestive of renal loss probably Distal RTA. ESR and CRP-q being normal with no ongoing inflammatory pathology. ANA by IF was sent which was 1:1000 with Speckled pattern. ANA Blot was sent which was strong positive for SSA, SSB and Ro52kD establishing a diagnosis of Sjogren's disease. She received potassium correction and oral sodium bicarbonate and currently on lifelong potassium without development of any sicca symptoms or joint involvement till as of date. Conclusion: Sjogren's syndrome, a connective tissue disorder related to impaired exocrine gland involvement and late onset joint involvement rarely presents as Hypokalemic periodic paralysis. Thus, it is paramount for clinicians to remember to rule out Sjogren's syndrome in case of hypokalemic periodic paralysis in middle age female group even in absence of sicca symptoms.
Keywords: Sjogren's disease, hypokalemiac periodic paralysis, distal rta, hypokalemia, SSA, SSB, Shohl's
Edition: Volume 13 Issue 6, June 2024,
Pages: 806 - 809