Treatment of Severe CNS Lupus with Intravenous Immunoglobulin

Design

Case presentation

A 20-year-old Hispanic woman, previously healthy, was admitted with hypertension, oliguria, and generalized anasarca, which has progressed over approximately 6 months. Labs showed the following: blood urea nitrogen 117 mg/dL, serum creatinine 9.8 mg/dL (estimated creatinine clearance 3 mL/minute), albumin 1.6 g/dL, hemoglobin 7.7 g/dL, and lactate dehydrogenase 2617 U/L. Results from blood smear suggested microangiopathy hemolytic anemia. Liver function tests were normal, with urinalysis showing hematuria, 4+ protein, urine protein excretion 3.8 g/24 hours, and red cell casts. Rheumatologic studies showed positive antinuclear antibodies (ANA), low complement C3, and low CH50. Chest x-ray revealed a left-sided pleural effusion. 

Hemodialysis was initiated, with severe chronic but active lupus glomerulonephritis diagnosis confirmed upon renal biopsy. Systemic lupus erythematosus (SLE) was diagnosed based upon presence of serositis, nephritis, hemolytic anemia with thrombocytopenia, and positive ANA. On day 5 of hospital admission, patient had generalized tonic-clonic seizure treated with phenytoin; head computed tomography (CT) scan results suggested hypertensive encephalopathy. Patient was treated with methylprednisolone 1 g/day for 3 days, followed by 1 mg/kg/day in divided doses but showed no response. Pleural effusions worsened and were treated on day 18 with cyclophosphamide 1,050 mg intravenously (IV). Further testing showed cerebral arterial irregularities, and patient had additional generalized seizure on day 21.

On day 23, CT results were consistent with ischemic changes secondary to central nervous system (CNS) lupus vasculitis; patient initiated on methylprednisolone IV boluses of 1 g/day for 3 days and intravenous immunoglobulin (IVIG) 400 mg/kg/day for 5 days. Hemiparesis improved, and repeat head magnetic resonance imaging scan on day 38 showed improvement in pleural and pericardial effusion sizes, hemolysis and thrombocytopenia resolution, and complement levels normalized. Patient discharged with dialysis on day 39 and continued corticosteroids at home. Dialysis was discontinued 2 months following with complete resolution of neurological symptoms seen at 6 months. 

Study Author Conclusions

Neuropsychiatric Lupus and Lupus Nephritis Successfully Treated with Combined IVIG and Rituximab: An Alternative to Standard of Care

Design

Case presentation

A 35-year-old black male patient with a history of hypertension and cardiomyopathy presented with symptoms including significant weight loss, fatigue, night sweats, and enlarged lymph nodes. Initial tests showed normocytic normochromic anemia, positive antinuclear antibody, and low C3 and C4 levels. A CT scan revealed enlarged lymph nodes and hepatosplenomegaly. After an axillary lymph node biopsy confirmed benign follicular and paracortical hyperplasia, a diagnosis of systemic lupus erythematosus was made. The patient was started on prednisolone 30 mg daily and hydroxychloroquine 400 mg daily. 

Six days later, he returned with severe musculoskeletal symptoms, including arthritis in multiple joints, and was admitted. Intravenous methylprednisolone 1 mg/kg was initiated, and he developed sepsis with Salmonella non-Typhi and methicillin-sensitive Staphylococcus aureus bacteremia. The patient required ICU admission, mechanical ventilation, and renal replacement therapy due to acute kidney injury. A kidney biopsy confirmed lupus nephritis (Class IV).

Despite extensive testing, including echocardiograms and imaging, no definitive valve vegetation or pulmonary embolism was found. However, CT imaging suggested septic emboli. The patient met the Duke criteria for infective endocarditis and was treated with meropenem 1 g IV TID for eight weeks. During his ICU stay, he developed neuropsychiatric symptoms, which were diagnosed as lupus-related, alongside myocarditis. Due to his severe sepsis, he was treated with intravenous immunoglobulin (IVIG) 0.4 g/kg/day for five days, rituximab 500 mg once, and pulse steroids 1 g/day for five days.

After treatment, the patient's condition improved significantly. His hallucinations subsided, and biochemical markers showed improvement. Three weeks later, the patient was started on mycophenolic acid 500 mg, gradually increased to 1 g BID. He continued prednisolone 1 mg/kg daily for four weeks, with a tapering dose reduced by 10 mg per week, and hydroxychloroquine 400 mg daily to maintain remission and manage other systemic manifestations.

Study Author Conclusions

Neuropsychiatric Systemic Lupus Erythematosus Presenting as Mania With Psychosis Treated Successfully With Intravenous Immunoglobulin: A Diagnosis of Associations and Exclusions

Design

Case presentation

A 22-year-old black female patient was involuntarily admitted to the psychiatry unit with a 2-week history of “unusual behavior,” including an elated mood, excessive energy, auditory hallucinations, and persecutory/grandiose delusions. She scored 37 on the Young Mania Rating Scale (YMRS). The urine drug screen was positive for cannabis, and her blood alcohol screen was negative. She had no personal or family history of psychiatric illness.

Upon admission, she was started on olanzapine for new-onset mania with psychotic features. Five months prior, during a hospitalization for a gastric ulcer, her anti-dsDNA level was found to be 288 IU/mL without further evaluation. Consequently, she was transferred to the medical service, and a consult service was engaged.

The patient was initially treated with prednisone 60 mg/day, hydroxychloroquine 200 mg/day, and azathioprine 50 mg/day but refused olanzapine. After 3 days with no change in the YMRS score, methylprednisolone 500 mg twice daily was added, but there was no improvement after 5 days, leading to the tapering of both methylprednisolone and prednisone. Two days later, intravenous immunoglobulin (IVIG) was administered at 2 g/kg over 2 days. Following IVIG therapy, her YMRS score decreased to 12 and 4, respectively, 1 and 2 days after completing IVIG. Four days post-IVIG, she was discharged with a YMRS score of 1. At a 2-month follow-up, she remained adherent to immunosuppressants with no relapse of neuropsychiatric systemic lupus erythematosus (NPSLE).

Study Author Conclusions