A Pseudo Guillain Barre Syndrome (GBS) Presentation of Sjogren’s Syndrome

Sir, Primary Sjogren’s syndrome (pSS) is a chronic autoimmune disorder affecting exocrine glands.[1] Renal involvement can be tubulointerstitial nephritis (TIN) and glomerular involvement, may present with secondary hypokalemic periodic paralysis (HPP) as the first manifestation of pSS.[1] Surprisingly, very severe weakness requiring mechanical ventilation or even sudden death, secondary to hypokalemia in distal renal tubular acidosis (dRTA) has been well described with pSS.[2] [Figure 1]. A similar clinical scenario “lai tai” was described in patients with endemic dRTA (EdRTA), in the North Eastern part of Thailand in 1991.[3]Figure 1: Pathogenesis of hypokalemia in Sjogren’s syndromeFour patients within the age range of 17 to 42 years presented to our healthcare facility over a span of 6 months with a similar clinical profile [Table 1] from a specific region of Hyderabad raising a possibility of endemicity of “Primay Sjogren’s Syndrome (pSS) with distal renal tubular acidosis (dRTA) presenting as severe quadriparesis secondary to hypokalemia.” Female preponderance with a F: M = 3:1. One of them had sicca symptoms and two of them had hypothyroidism. All the four had neck power weakness and two of them were on mechanical ventilation. However, all of them recovered completely and were initiated on long-term immunomodulation and potassium supplements.Table 1: Clinical features and laboratory parameters of the patients enrolled in the studyThere is limited literature about respiratory and bulbar muscle involvement in HPP, authors postulated that progress for respiratory arrest is influenced by interindividual differences in sensitivity toward hypokalemia and transmembrane K gradient of respiratory muscle.[2] Recognition of this serious clinical entity is very important as symptomatic treatment can be 100% effective.[4] Treatment protocols depend upon target organ involved. In renal involvement, based on the European League Against Rheumatism Sjögren’s syndrome disease activity index (ESSDAI), mild disease is symptomatically treated with potassium replacement and alkali correction of the metabolic acidosis, moderate disease is treated with glucocorticoids and other oral immunosuppressant agents, and severe disease is treated with glucocorticoids as first line and rituximab or cyclophosphamide as second line agents and plasma exchange as a rescue therapy.[4] We propose a hypothesis of endemic dRTA, which is associated with Sjogren’s syndrome with secondary hypokalemic periodic paralysis presenting with a unique pattern of severe muscle weakness and impending respiratory failure closely mimicking GBS. In view of an autoimmune etiopathogenesis, patient’s response to intravenous immunoglobulin (IVIG) adds more confusion and resemblance to GBS. A few important clues for making an accurate diagnosis are: severe hypokalemia (<2 meq/L) at presentation, rapidly progressive areflexic quadriparesis with bulbar and respiratory muscle involvement, history of sicca symptoms, and response to steroids. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.