Table 1. Ten case series of primary Sjögren’s syndrome with renal tubular acidosis and central pontine myelinolysis.
| Bruns et al. [23] | Abdulla et al. [24] | Maturu et al. [8] | Nagashima et al. [25] | Saxena et al. [26] | KH Yoon et al. [10] | Watson et al. [27] | Rubo, S et al. [9] | Index Case | |
|---|---|---|---|---|---|---|---|---|---|
| Germany | India | India | Japan | India | Singapore | London | China | South Africa | |
| Age | 17 | 28 | 33 | 42 | 45 | 47 | 64 | 75 | 42 |
| Sex | Female | Female | Female | Female | Female | Female | Female | Female | Female |
| Sicca symptoms | No | Yes | - | Yes | Yes | No | Yes | Yes | Yes |
| Diagnosis type I RTA | On index CPM admission | Occurred prior to CPM diagnosis | Occurred 1 year after CPM diagnosis | On index CPM admission | On index CPM admission | Occurred prior to CPM diagnosis | On index CPM admission | On index CPM admission | On index CPM admission |
| On presentation | Muscular weakness with leg pain. Later developed bilateral 6th nerve palsies, dysarthria and dizziness; Additional Hashimoto thyroiditis, vitiligo, celiac disease |
Jaundice, hepatic encephalopathy, generalized tonic clonic seizures; Additional hepatitis A infection |
Sudden onset, rapidly progressive quadriplegia, severe dysarthria, bilateral facial palsies, bulbar palsy | Periodic weakness from hypokalemia for 9 years prior to index presentation | Progressive weakness of all 4 limbs, difficulty with respiration and swallowing, dysarthria and altered sensorium | Obtunded with quadriplegia; previous history of hypokalemic weakness and small vessel vasculitis of the bowel | 3-month history of fatigue, nausea and vomiting, hypercalcemia; later developed bilateral 6th nerve palsies with generalized weakness | Rapid, progressive quadriplegia, hypersomnia, dysphagia | Progressive global quadriplegia, ophthalmoplegia, and encephalopathy |
| Admission serum sodium | – | 137 mmol/L | Hypernatremia; no history of hyponatremia with rapid sodium correction | – | Hypernatremia | Hypernatremia (154 mmol/L) |
Reported normal | – | 140 mmol/L |
| Admission serum potassium | 1.8 mmol/L | 2.6 mmol/L | 2.2 mEq/L | Low | 1.9 meq/L | Low | 2.0 mmol/L | 1.4 mmol/L | 1.6 mmol/L |
| ANA | Positive | Positive | – | – | Positive | Positive | Positive | Positive | Positive |
| Anti SSA +/Anti SSB + | Anti SSA +/anti SSB + | Anti SSA +/anti SSB + | Anti SSA +/anti SSB + | Anti SSA +/anti SSB - | Anti SSA +/anti SSB - | Anti SSA +/anti SSB– | Anti SSA +/anti SSB– | Anti SSA +/anti SSB + | Anti SSA +/anti SSB + |
| Serum IgG and IgA | – | – | – | – | – | Elevated | – | – | Elevated |
| Salivary gland biopsy | – | – | Yes | Yes | – | – | – | – | No |
| Treatment | Electrolyte and acid-base balance correction, steroids | Electrolyte and acid-base balance correction | Electrolyte and acid-base balance correction, steroids | – | Electrolyte and acid-base balance correction, steroids, CYC | Steroids, CYC, and IVIG; After relapse required PE and IVIG |
Electrolyte and acid-base balance correction, hydroxychloroquine | Electrolyte and acid-base balance correction, steroids, CYC | Electrolyte and acid-base balance correction, steroids, CYC |
| Short-term neurological outcome | Response seen within 2 weeks: by 4 weeks the muscle weakness had resolved |
Neurology improved within 1 month | Within a week, motor power recovered | – | Within 2/52 recovery to walking | Complicated course post-treatment – neutropenic sepsis, PE; Able to ambulate independently at 1 month |
Good neurological recovery | Over several days: LOC and cranial muscle strength improved; within 2 weeks, neurology significantly improved |
Improved neurology within days |
| Long-term neurological outcome | Occasional dizziness, 6th nerve palsies persistent | Complete neurological recovery | Asymptomatic at 6 months | – | Residual right lateral rectus palsy | Recurrent relapsing disease | Persistent diplopia | Mild residual truncal ataxia | Complete neurological recovery |
CPM = central pontine myelinolysis; Anti-SSA = anti-Sjogren’s syndrome-related antigen A autoantibodies; Anti SSB = anti-Sjogren’s syndrome-related antigen B autoantibodies; CYC = cyclophosphamide; IVIG = intravenous immunoglobulin; PE = plasma exchange; LOC = level of consciousness.