Lupus Nephritis or Infections—Whom to Blame?

Abstract

A 20-year-old woman was admitted with pedal edema, facial puffiness of 5-6 months duration, along with additional complaints of alopecia, oral ulcers, and photosensitivity. There was no arthralgia/ arthritis or Raynaud's phenomenon. On clinical examination and laboratory investigations, she was found to be anemic (Hb-6.8 mg/ dL) with pancytopenia (TLC-2,800/μL, platelet count-119,000/ μL). She was having nephrotic range proteinuria (24-hour urinary protein-3.2 g/TV) and mild renal dysfunction (S. creatinine-1.3 mg/dL). Her serum complement (C3/C4) was low. Based on the above findings, a diagnosis of systemic lupus erythematosus (SLE) was made with hematological and renal involvement. This was further supported by positive ANA and dsDNA titers. Her pANCA was also positive. Hence, a renal biopsy was performed for histopathological examination which confirmed the presence of crescentic glomerulonephritis due to lupus nephritis (LN) (class IV) along with thrombotic microangiopathy (TMA). She was given MP pulses and oral steroids (1 mg/kg/day). Cell counts improved after steroid treatment. Thereafter, a monthly cyclophosphamide pulse was given according to NIH protocol with oral steroids. Two weeks later, she complained of depressive symptoms. Her renal function deteriorated with rising serum creatinine (4.8 mg/dL) and blood urea (116). Her platelet count dropped to 78,000/mL and serum LDH was high (2,200 IU/L). A possibility of TTP/CNS lupus was considered. She was managed with anti-depressants, seven sessions of plasma exchange, and another dose of endoxan in view of worsening of disease activity. Neuropsychiatric symptoms improved and serum creatinine came down to 3.3 mg/dL. However, after a few days, she became febrile with pain and swelling of the left thigh, decreased oral intake, and irritability. A possibility of left thigh cellulitis was considered and managed with piperacillin and tazobactam in emergency OPD. She subsequently became afebrile with a reduction in redness and swelling. Despite this, she remained irritable and had decreased oral intake. Thus, she was readmitted for the third time.