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Systemic lupus erythematosus is an autoimmune disease, which presents a variety of clinical and histological manifestations in different organs.
Currently, information has been collected from kidney biopsies that detect structural lesions that affect the functionality of the organ through immunofluorescence and electron microscopy. An association with other atypical immunological diseases has been observed, such as lupic podocytopathy, thrombotic microangiopathy, interstitial nephritis, cryoglobulinemic glomerulonephritis, acute tubular necrosis and vasculitis.
Although these associations of immunological diseases are difficult to diagnose, leading us to perform ineffective treatment and increasing the high rate of morbidity and mortality.
22-year-old female patient; History of maturational delay; Recurrent urinary tract infections; Hypothyroidism.
Clinical picture: Tonic-clonic convulsive episodes on 3 occasions, physical examination presents left parietal cephalohematoma, edema in 2/6 lower limbs, hypertension and anuria are noted.
Laboratory: Normocytic normochromic anemia, Urea 213 creatinine 10.2, K 4.7 P 10.6 Gasometry: Metabolic acidosis and hyperlactacidemia. ESR 80 CRP 176
Immunology: Electrophoretic proteinogram: Hypoproteinemia, Hypoalbuminemia, hypocomplementemia, FAN homogeneous pattern, anti DNA, ENAS antiRo positive, ANCA P-PR3 and antiMBG positive.
Urinary sediment: Optical microscopy dysmorphic red blood cells 30% and acanthocytes 5%.
Renal Ultrasound, Right Kidney cyst in the upper pole measuring 18mm, both kidneys with loss of corticomedullary relationship.
Echocardiogramleft ventricular hypertrophy, ventricular ejection fraction 60%.
Negative neuroimaging for injury.
Chest CT with moderate pleural effusion and mild pericardial effusion.
PBR: MO 10 glomeruli 5 with global sclerosis 4 with cellular crescents, one of them circumferential type, it coexists with segments of capillary collapse and segmental sclerosis and capsular adhesion, the third glomerulus with a fibrous crescent, in all of them an increase in the matrix and mesangial cellularity, global and diffuse mild regular thickening of focal leukocyte capillary walls and diffuse podocyte damage. Tubuloinsterstitial involvement of 17%. Negative IFI.
It is interpreted as SLE associated with ANCA Vasculitis with glucocorticotherapy + immunological treatment (cyclophosphamide plus mycophenolate mofetil), plus dietary measures with a good response, achieving recovery of partial diuresis.
Given that the clinical presentation of SLE is heterogeneous in our case, the biopsy findings and for clinicians are characteristic to define its association with Vasculitis, this being a challenge for the doctor and demonstrating the complexity of the management of rare entities of associated immunological pathologies. .A delay in diagnosis leads to serious complications that affect the patient's quality of life, which can cause irreversible damage, increasing morbidity and mortality.