A 45-year-old man complained of general malaise two months before hospitalization, hypoxia, asthenia, adynamia, moderate morning pulsatile global headache and loss of visual acuity (3 kg).
Upon review by systems, the patient reported nausea, occasional vomiting, polydipsia, polyuria and foamy urine.
As antecedents, Guillain's syndrome was interested in chronic dialysis (two episodes: years 2000 and 2006), systemic arterial hypertension diagnosed two months before, former smoker up to two years of 5 cigarettes per day and terminally ill father;
Physical examination revealed regular general conditions, with temperature of 37oC, blood pressure of 159/100 mm Hg, respiratory rate of 20 per minute, and heart rate of 100 per minute.
There were no other positive findings.
Paraclinical tests are summarized in Table 1.
Renal ultrasound and two-dimensional color Doppler echocardiography were normal, and chest X-ray showed signs of mild pulmonary dyssynthetic hypertension.
1.
Presumptive diagnoses included nephrotic or nephritic syndrome, uncontrolled systemic arterial hypertension and acute renal failure.
Re-adjustment of antihypertensive therapy, thromboembolic prophylaxis, antiemetics, gastroprotection and hyposodic diet were initiated.
One day after admission, clinical improvement was observed paulatin and tests for glomerular disease were ordered.
Ten days after the findings, a percutaneous renal biopsy was performed, in which the following was observed: number of glomerulopathies and capillary refferent ducts (diverse hymangiomas); the mean number of arterioles + focal segmentation;
With immunofluorescence we found: linear IgG (++) capillary basement membrane; IgM (++) vascular wall; C3 (+) some vascular walls; light chain kappa (+++) light tubular basement membrane, basement membrane.
Frequently fixed electronic contour was found: diffuse thickening of the capillary membrane with presence of double lumens and interposition of mesoid deposits+dense capillary membranes, with appearance of monolayer deposits, fam
Anatomopathology diagnosed menoproliferative pattern nephropathy, disease type by dispositif of light chains kappa.
1.
Almost one month after percutaneous renal biopsy, the patient was admitted to the emergency department due to hypertensive emergency, worsening of weight loss, anaemia (hemoglobin level of 11 g/dl), asthenia,
Given the result of renal biopsy, assessment began.
Bone marrow biopsy showed 70% cellularity, relationship between myeloid and erythroid 3:1, and absence of erythrocytes.
There was representation of the hematopoietic lines, of normal appearance, number and location.
No fibrosis or colitis was observed.
Protein electrophoresis was normal in serum and showed no monoclonal bands in urine.
Serum levels of light chains were 1.64 mg/dl (1.38-3.75) for kappa and 0.52 mg/dl (0.93-2.42) for lambda.
The bone study showed no lytic lesions.
Serum immunoglobulins were normal.
Increased levels of beta 2 microglobulin were found.
The following studies were also performed: gastric, colon and rectal biopsy, with no evidence of malignancy or amyloid deposit; subcutaneous abdominal fat aspiration biopsy, negative with Congo red staining; chronic electromyopathy; neuroconduction.
The patient progressed to a rapidly progressive renal failure, with uremic symptoms and signs of hypervolemia (two months after the first hospitalization) and entered a renal replacement therapy program.
