Case Report: SLE Flare-up during Pregnancy

Abstract

SLE is a chronic autoimmune disease where the immune system attacks the body’s own tissues. And Castleman disease is a rare lymph node disorder characterized by abnormal lymph node growth. Systemic Lupus Erythematous (SLE) is a chronic, systemic autoimmune disease in which the body’s immune system produces autoantibodies that attack its own tissues, causing inflammation and damage to multiple organs.
Case Presentation: this case report describes 29 years old female G3P1L1A1 admitted in AIIMS with chief complains of neck swelling and shortness of breath for 5 days, oral ulcer, fever and loose stools for 4 days. Patient was asymptomatic 5 days back when she developed loose stools, associated with abdominal pain and tenesmus which resolved spontaneously. From last 5 days she had worsening SOB from m MRC I to IV associated with orthopnea, since last 2 days she had fever, 103F, associated with chills and rigors, relieved on antipyretics. Patient had history of progressive neck swelling over 5 days, bilateral shoulder joint, thigh and small joints, alopecia, and Raynaud phenomenon.
Obstetric History: G3P1L1A1, LMP 10/5/2023, last child birth 7 years back, normal vaginal delivery at 9-month POG without any antepartum and postpartum complications.
Management and Outcome: The patient presented with the aforementioned complaints at AIIMS. In view of type 2 respiratory Failure NIV was started. Routine investigations revealed low C3/C4 levels, elevated CK. increased 1011 and signs of transaminitis. Suspecting a flare-up of Systemic Lupus Erythematous (SLE) and myositis, the patient received a 500 mg dose of Methylprednisolone pulse daily for 3 days (July 21st -July 23rd) and 2.4g/day of IVIG for 5 days (July 21st July 26th). Due to worsening respiratory distress and NIV failure, the patient underwent elective intubation and was transferred to ICU for intensive monitoring. Patient developed Ventilator-Associated Pneumonia (VAP), and was empirically started on Polymyxin B and Minocycline, later modified to Cefoperazone and sulbactam based on culture report. In order to address difficulty in weaning, and to prevent further ventilator-associated pneumonia, a tracheostomy was performed.
The patient experienced three episodes of unconsciousness lasting for 2 hours each, suspecting seizures, an EEG and brain MRI were conducted, both of which yielded normal results. Following the administration of pulse steroids and IVK. The patient was started on Prednisolone (50 mg) and HCQ (200 mg). Patient developed Non-Anam Gap Metabolic Acidosis (NAGMA) with hypokalemia, suggestive of Renal Tubular Acidosis, likely due to underlying connective tissue disease. Routine urine culture indicated enterococcus farsis infection, for which single dose Fosphomysin was administered. Myositis panel revealed antibodies against Mi22, SRP and PM SCL were positive. A whole-body muscle MRI indicated increased STIR signal intensity in the lower limb muscles: Gradually, the patient's condition improved, leading to successful weaning off the ventilator and Decannulation of the tracheostomy site an ultrasound for foetal well-being confirmed a single, live intrauterine pregnancy. The case was discussed with Obstetrics and Gynecology consultant regarding the potential use of Rituximab and its implications for the ongoing pregnancy. Following consultation with Dr. Siddhartha Jain, it was advised that as the patient's disease activity was improving and she expressed willingness to continue the pregnancy, the administration of Rituximab might not be advisable with addition of Azathioprine 50mg once daily with tapering Wysolone. An early level 2 scan was recommended. Currently CRP, LDH, CK, C3 and C4 are in normal range. Patient is hemodynamically stable and planned for discharge with following vitals.
Conclusion: This case highlights the complexity of managing SLE with multi-system involvement during pregnancy, with features suggestive of lymphoproliferative disorder. Early diagnosis, aggressive immunotherapy, and multidisciplinary care are essential for favorable outcomes.