SUCCESSFUL LONG-TERM MANAGEMENT OF LUPUS NEPHRITIS WITH DISSEMINATED RENAL AND GASTROINTESTINAL CRYPTOCOCCOSIS: A CASE REPORT

Introduction:

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by multi-organ involvement, including lupus nephritis, a serious renal manifestation that often requires long-term immunosuppressive therapy. While essential for controlling disease activity, such therapy increases the risk of opportunistic infections, particularly cryptococcosis. Cryptococcosis, caused by Cryptococcus species, commonly affects the central nervous system and lungs in immunocompromised patients. However, disseminated cryptococcal infections involving the kidneys and gastrointestinal tract are exceedingly rare in SLE patients, creating a unique clinical challenge in balancing effective immunosuppression for lupus control and antifungal treatment for the infection.

Methods:

Case Presentation:

A 36-year-old gentleman, an insurance agent by profession and a known case of lupus nephritis diagnosed one year ago, presented with generalized swelling, decreased urine output, fever, and shortness of breath. He had previously been on corticosteroids and mycophenolate mofetil for 6 months but discontinued treatment on his own. On clinical evaluation, he was found to have anemia, renal dysfunction, hypoalbuminemia, proteinuria, microscopic hematuria, and low complement (C3 and C4) levels. Imaging revealed centrilobular nodules, consolidation, and pleural effusions in the lungs, suggestive of a superimposed lung infection. Abdominal ultrasound showed altered liver echotexture, raising the suspicion of chronic liver disease, likely of autoimmune etiology. A renal biopsy confirmed active lupus nephritis, but an incidental finding of a fungal granuloma consistent with Cryptococcus was also identified. An endoscopy done to rule out varices revealed duodenal nodules, and histology of the biopsy confirmed disseminated cryptococcosis.

Treatment:
The treatment approach required a careful balance of immunosuppression to control lupus nephritis and antifungal therapy to address disseminated cryptococcosis. Following a literature review, two case series of SLE patients with cryptococcosis treated with immunosuppression were identified to guide treatment. The patient was started on prednisone (1 mg/kg) and antifungal therapy with amphotericin B lipid complex (5 mg/kg/day) for 8 weeks, followed by fluconazole (800 mg/day) as consolidation therapy. Long-term fluconazole (200 mg/day) was continued as maintenance therapy in line with the 2010 IDSA guidelines for disseminated cryptococcosis. Corticosteroids were gradually tapered over 3 months, and proteinuria was managed with ARBs and SGLT2 inhibitors.

Results:

Follow-Up:

The patient was followed monthly. After 8 months, a repeat endoscopy with biopsies revealed that the gastric mucosa was free of cryptococcus, though residual infection persisted in the duodenal mucosa, warranting continued antifungal therapy. Renal function stabilized with improved creatinine levels and reduced proteinuria, and the patient remained stable on maintenance fluconazole therapy.

Discussion:
This case underscores the complexity of managing disseminated cryptococcosis alongside active lupus nephritis. Patients with SLE are at high risk for opportunistic infections due to intrinsic immune dysfunction and long-term use of immunosuppressive therapies, including corticosteroids, mycophenolate mofetil, and cyclophosphamide. Cryptococcal infections in SLE patients typically affect the central nervous system and lungs, but renal and gastrointestinal involvement is extremely rare. Managing both lupus nephritis and cryptococcosis simultaneously requires a multidisciplinary approach that balances the need for immunosuppression with the risk of exacerbating the infection. In this case, the patient’s treatment included corticosteroids and antifungal therapy with amphotericin B, followed by fluconazole for maintenance. The patient’s renal dysfunction required careful monitoring and frequent adjustments to the therapy.

Conclusions:

This case illustrates the delicate balance required when managing SLE flares with concomitant opportunistic infections like cryptococcosis. The successful long-term management of this patient involved antifungal treatment alongside carefully titrated immunosuppressive therapy to control lupus nephritis without exacerbating the infection. The patient's one-year follow-up showed good renal recovery, with only residual duodenal cryptococcal infection, underscoring the importance of ongoing monitoring and maintenance therapy in preventing recurrence and achieving favorable outcomes in such complex cases.

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.