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Lupus podocytopathy (LP) represents 1% of lupus nephritis (LN) biopsies. Focal segmental glomerulosclerosis (FSGS) in Systemic Lupus Erythematous (SLE) is rare, with nearly 20 reported cases. Histological transition occurs after several relapses, evolving into LN class IV or V. Diagnosis of SLE precedes or is simultaneous with FSGS, usually with low complement and positive ANAs and dsDNA.
We present 2 cases LP FSGS-type with recurrent Nephrotic Syndrome (NS) and transition to LN class V.
Case 1: A 31-year-old woman experienced arthritis and NS 6 years ago. The first renal biopsy (RB) showed tip-variant FSGS with mesangial deposits in immunofluorescence (IF) and electron microscopy with >50% obliteration of podocyte processes without immune complexes (Fig.1). Treatment with prednisone (PDN) at 1 mg/kg/d was initiated. After 6 weeks (wks) complete remission (CR) was achieved and PDN was reduced. After 5 months (mo), the first relapse (RL) of NS occurred. PDN was increased and Tacrolimus was added leading to CR in 12 wks. After 1 mo of maintenance therapy (MT) she had a second RL with low complement and positive ANAs. Second RB showed the same findings as the first one. PDN was increased, with CR achieved within 3 wks. MT was given for 8 wks, but a third RL occurred with low complement and positive ANAs, anti-SM and anti-RNP. Mycophenolate mofetil (MMF) was added and PDN was increased, achieving partial remission at 8 wks. During PDN reduction, a fourth RL occurred. The third BR showed LN class V (Fig.2). Methylprednisolone (MTP) and monthly cyclophosphamide (CFM) were initiated. Prior to the 4th dose, she experienced a fifth RL. MTP and Rituximab (RTX) (4x1g) were given, resulting in CR at wk 4. MT continued with PDN and MMF. At 6 mo, with normal serological pattern, a fourth BR was performed, revealing cicatricial FSGS with negative IF. Twelve mo after RTX, PDN was stopped and MMF was reduced. To demonstrate histological remission, a fifth BR was performed showing cicatricial FSGS with negative IF. MMF was withdrawn and she continued with hydroxychloroquine (HCQ) and enalapril. The latest assessment after 2 mo showed CR.
Case 2. A 32-year-old man with a history of alcoholism and drug abuse presented with NS, hypertension, and acute kidney injury, requiring hemodialysis. Viral infections were ruled out. The RB showed collapsing glomerulopathy with negative IF (Fig.3), meeting diagnostic criteria for SLE and LP. He was started on MTP (3x1g) and CFM (500mg), leading to recovery of kidney function and proteinuria reduction. He continued MT with HCQ and PDN for 6 wks achieving CR. Due to non-adherence to treatment, he abruptly stopped PDN after 3 mo and restarted drug use. At follow-up, a slight increase in proteinuria was shown, along with elevated ANAs, dsDNA, anti-Ro, anti-nucleosomes and low complement. A second RB confirmed LN class III+V (Fig. 4). MTP (3x500mg) and RTX 1 g every 2 wks was given. Currently the patient is in remission.
Conclusions
The histological transition in serial biopsies in proliferative LN has been rarely reported. The cases we report are noteworthy for their positivity for anti-Sm, anti-RNP, anti-nucleosomes and anti-Ro. In addition to the high PDN dependence, requiring more aggressive treatment to achieve remission.