Unmasking Renal Disease in Systemic Lupus Erythematosus: Beyond Lupus Nephritis.

A 64-year-old Caucasian woman with a history of hypertension and systemic lupus erythematosus (SLE) was referred to a nephrology clinic due to persistent microscopic hematuria and trace proteinuria. Initial tests showed elevated antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA), and anti-Sjögren's syndrome-related antigen A (anti-SSA) levels, while other markers remained within normal limits. Over the course of a year, her urine protein-creatinine ratio increased, prompting a renal biopsy. The biopsy revealed focal crescent formation in some glomeruli and mild segmental mesangial hypercellularity in others. Although the possibility of antineutrophilic cytoplasmic antibody (ANCA)-associated nephritis with superimposed IgA nephropathy was considered, negative myeloperoxidase and proteinase 3 antibody tests led to a final diagnosis of IgA nephropathy. The patient's treatment included adding prednisone to her existing valsartan prescription for hypertension, which resulted in improved proteinuria. SLE is an autoimmune disease that can cause chronic inflammation and damage to vital organs. Approximately 50% of SLE patients may experience lupus nephritis (LN), underscoring the importance of urinalysis and renal function tests. This case presents a female patient with SLE and IgA nephropathy, a rare association that requires distinction as it affects disease management. IgA nephropathy is the most common cause of idiopathic glomerulonephritis and can lead to end-stage kidney disease in around 40% of cases. A renal biopsy is also crucial for diagnosing IgA nephropathy in patients with or without another autoimmune disease. Focal crescent formation, a histological feature observed in this case, helped exclude several diagnoses, such as lupus nephritis or pauci-immune glomerulonephritis. The primary goal of treating IgA nephropathy is to prevent disease progression. Initial treatment includes controlling blood pressure, reducing proteinuria, and implementing lifestyle modifications. Corticosteroid therapy may be considered if supportive care is insufficient.

Catastrophic Antiphospholipid Syndrome Presenting as Congestive Heart Failure in a Patient with Thrombotic Microangiopathy.

Thrombotic microangiopathic syndromes are characterized by thrombus formation leading to microangiopathic hemolytic anemia, thrombocytopenia, and end-organ injury that most often affects the kidney and brain. Patients with thrombotic microangiopathy can also present with cardiac involvement, which has been shown to worsen their prognosis. We describe the case of a 46-year-old woman who presented with acute congestive heart failure as a manifestation of catastrophic antiphospholipid syndrome, which is characterized by rapidly progressing multiorgan involvement. Targeted therapy improved our patient's cardiomyopathy and saved her life. Increased recognition of thrombotic microangiopathy as an underlying pathophysiologic mechanism in heart failure and initiation of timely treatment may help to prevent death in patients with thrombotic microangiopathy.

The enigma of vasculitis.

We describe the case of an 86-year-old man presenting with clinical symptoms suggestive of Temporal Arteritis. The evolution of the case prompted extensive work-up, including temporal artery and kidney biopsy. Based on the clinical and pathological findings, a diagnosis of ANCA-negative granulomatous necrotizing vasculitis involving the small-, medium-, and large-size vessels was made. The patient was treated with prednisone and cyclophosphamide, which was later switched to rituximab. The patient remained asymptomatic under this regimen and stabilization of his kidney function was achieved.