NEPHRO-CALYPSE: A RARE CASE OF EVANS SYNDROME CO-EXISTING WITH THROMBOTIC MICROANGIOPATHY PRESENTING AS RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS IN A 31-YEAR-OLD FILIPINO FEMALE

Rapidly Progressive Glomerulonephritis (RPGN) is a clinical syndrome of a rapid loss of kidney function, commonly presenting with acute glomerulonephritis. Due to its rarity affecting only 7 to 10 per 1 million population, it is important to discuss as a delay in treatment will lead to rapid progression to end-stage renal disease. Evans Syndrome is a rare autoimmune disorder characterized by autoimmune hemolytic anemia and immune thrombocytopenia, with or without immune neutropenia. On the other hand, Thrombotic Microangiopathy (TMA) is a pathologic process manifesting as microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage caused by generalized microvascular thrombosis. Incidence on the co-existence of Evans Syndrome and TMA is exceptionally rare that it poses diagnostic and therapeutic challenge. We present a case of a 31-year-old female with RPGN caused by immune-mediated hemolytic and renal syndromes.

We present a case of a 31-year old female who complained of a 1-month history of easy fatiguability, lower back pain, and anorexia. She denies history of skin rashes, altered urine color, urinary symptoms, fever, upper respiratory tract infections, photosensitivity, joint pains, ulcers, and hemoptysis. Her baseline renal function was normal, including her urine examination. The patient was admitted one month as a case of RPGN and was scheduled for kidney biopsy. She was managed with Methylprednisolone pulse therapy and was discharged with tapering of her corticosteroids. On re-admission due to persistence of easy fatiguability, she was noted to have elevated blood pressure, had pallor, pale conjunctivae, and anasarca. 

She had elevated creatinine of 16.7 mg/dL (eGFR 3 mL/min/m3), normocytic normochromic anemia with hemoglobin of 4 g/dL, while her urinalysis showed pyuria, proteinuria and dysmorphic hematuria. She had a low PLASMIC score of 1 during this time.  Diagnostic work-up for RPGN revealed negative for hepatitis B and C, ANA, anti-dsDNA, anti-Sm, anti-RNP, p- and c-ANCA, and C3. However, during her course of stay, she developed thrombocytopenia, and her anemia had been persistent despite transfusion of packed red blood cells. Further workup revealed she had elevated Lactate Dehydrogenase, and a positive direct Coomb’s test. She was seen by the hematology service and was managed as a case of Evans Syndrome. Her peripheral blood smear showed schistocytes consistent with her renal biopsy, revealing Thrombotic Microangiopathy compatible with Malignant Nephrosclerosis, acute tubular injury, severe interstitial fibrosis and tubular atrophy. Immunostaining was negative for IgA, C1q, and C3. Her corticosteroid therapy was continued, and the patient is on hemodialysis thrice weekly. 

This case highlights complications of immune-dysregulation and endothelial injury. The co-existence of both Evans Syndrome and TMA presenting as rapidly progressive glomerulonephritis is rare, underlining the scale of immune complex deposition and complement activation triggered by immune cytopenias of both syndromes.  Early recognition and aggressive corticosteroid therapy are vital in the management to halt the development of end-stage renal disease. This presentation also highlights the importance of prompt multidisciplinary management for combined diagnosis and management.