Introduction:
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by its heterogeneity and potential for severe organ involvement. While most patients with SLE experience mild to moderate disease activity, a subset of patients develop life-threatening complications.
Methods:
A case of severe systemic lupus erythemtosus in nephrology departement of CHU sahloul
Results:
A 22-year-old female was diagnosed with systemic lupus erythematosus (SLE) based on positive antinuclear antibodies (ANA) at a titer of 1/3200 (anti-DNA+, anti-nucleosome+) and involvement of the joints and skin. She was treated with hydroxychloroquine and corticosteroids. The patient presented with a high SLE Disease Activity Index (SLEDAI) score, fever, alopecia, purpura, polyarthralgia, hemolytic anemia, and rapidly progressive glomerulonephritis (creatinine levels from 87 μmol/L to 447 μmol/L with nephrotic syndrome (albumin 27 g/L, total protein 3.8 g/24h)).
Cardiac involvement was evident with pericarditis, myocarditis, and impaired left ventricular function. The patient was started on an angiotensin-converting enzyme inhibitor (ACEI) and a beta-blocker.
On the digestive level, the patient experienced uncontrollable food vomiting with significantly elevated amylase and lipase levels. An upper gastrointestinal endoscopy showed mild chronic antral and fundic gastritis. A pancreatic MRI revealed features of edematous-interstitial pancreatitis, stage C according to Balthazar classification. A hepatobiliary MRI showed a liver with chronic hepatopathy associated with signs of intrahepatic biliary cholangitis.
The patient received three boluses of methylprednisolone followed by a maintenance dose of 1 mg/kg/day and mycophenolate mofetil (MMF).
The patient's condition deteriorated, requiring transfer to the intensive care unit for respiratory distress.
Despite receiving initial immunosuppressive therapy with corticosteroids and mycophenolate mofetil, followed by a switch to intravenous cyclophosphamide, the patient developed acute respiratory distress syndrome and refractory septic shock, ultimately leading to a fatal outcome.
This case illustrates the severity of systemic lupus erythematosus in a young 25-year-old woman with involvement of the skin, joints, kidneys, heart, and pancreas.
Conclusions:
This case report highlights the challenges associated with managing patients with severe, multisystemic flares of SLE. The rapid progression of disease and the refractoriness to standard therapies underscore the need for further research into novel therapeutic strategies for patients with fulminant lupus. Future studies should focus on identifying biomarkers that can predict disease flares and developing targeted therapies to prevent organ damage and improve patient outcomes.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.