The challenges of managing distal renal tubular acidosis in pregnant patients with primary Sjögren’s disease

Abstract

We present a case of a pregnant woman in her 40s diagnosed with primary Sjögren’s disease (pSjD) complicated by distal type I renal tubular acidosis. At 12 weeks’ gestation, she exhibited dry eyes, dry mouth and renal dysfunction for the first time. Electrolyte studies confirmed hyperchloraemic normal anion gap metabolic acidosis with elevated urine pH. Positive serologies included +ANA, +SSA and +RF. Schirmer’s test was positive. Renal biopsy indicated chronic tubulointerstitial nephritis with 40% interstitial fibrosis. Hydroxychloroquine and prednisone (60mg daily) improved renal function, but rapid tapering led to mild worsening in renal function, sicca symptoms and fetal growth restriction. Steroid dose escalation and azathioprine initiation led to clinical improvements. At 35 weeks’ gestation, she delivered preterm due to pre-eclampsia and intrahepatic cholestasis. The neonate had cutaneous neonatal lupus but was otherwise healthy. This case highlights the clinical complexity of managing distal renal tubular acidosis in pregnant patients with pSjD and the need for evidence-based treatment guidelines.