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IgA Nephropathy Case discussion

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by

Shaban Mohsen

on 26 November 2012

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Transcript of IgA Nephropathy Case discussion

Shaaban M. Ahmad Follow The Leads
68-year-old man presented with :

midabdominal, dull, postprandial pain that had persisted for 3 hours, preceded by nausea.
Past History Patient Presentation What's your work up ?? Work up.. CBC
Hemoglobin, 7.7 g/dL "2yrs ago it was 13 g/dl !!"
hematocrit, 21.0%
MCV 89.5 fL
leukocyte count, 6.7 × 109/L
platelet count, 151 × l09/L
Which one of the following is the least likely cause of this patient's anemia?
a.Ruptured abdominal aortic aneurysm
b.Cancer of the colon
c.Peptic ulcer disease
d.Hemolysis caused by the prosthetic aortic valve
e.Renal failure Work up .. Hypertension
coronary artery disease,
aortic stenosis.
two-vessel coronary artery bypass grafting
Aortic valve replacement 3 years earlier, followed by long-term coumarin therapy.
He also had a 50-pack-year history of smoking. Vital signs Mild tenderness over the epigastrium on deep palpation.
No rebound tenderness.
Bowel sounds were present,
No masses were palpated.
Rectal examination disclosed no notable findings.
On Examination BP 159/73 mm Hg
Pulse 72 beats/min
Respirations 16/min
Temperature, 36.7°C. 68-year-old man presented with :

Midabdominal, dull, postprandial pain that had persisted for 3 hours, preceded by nausea.
PT 21.5 seconds (normal, 8.4 to 12.0);
INR 2.8
Creatinine 4.1 mg/dL "2 yrs ago it was 1.4 mg/dl" !!
BUN 62 mg/ dL
LDH 240 U/L (normal, 112 to 257);
Amylase 48 U/L (normal, 35 to 115);
Total bilirubin 1.0 mg/dL
Alkaline phosphatase (ALKP) 138 U/L (normal, 98 to 251);
aspartate aminotransferase (AST), 51 U/L (normal, 12 to 31);
haptoglobin, 215 mg/dL (normal, 40 to 300).
Cancer of the large bowel frequently occurs in this age-group and can manifest with insidious blood loss. Abdominal pain (particularly epigastric) is a common symptom in peptic ulcer disease, which is often complicated by hemorrhage, especially in the presence of long-term anticoagulation. Anemia attributable to lack of erythropoietin production in renal failure should be considered a possibility in view of the patient's long history of hypertension. Prosthetic valve-associated hemolysis is the least likely cause of anemia in this patient because of lack of evidence of hemolysis, as indicated by normal LDH, bilirubin, and haptoglobin levels. Hemolytic anemia occurs more commonly in prosthetic mitral valve recipients than in aortic valve recipients and is associated with a decreased level of serum haptoglobin. With a history of cigarette smoking and atherosclerotic disease, this patient has an increased risk for development of an abdominal aortic aneurysm (AAA). Acute aneurysmal ruptures that are usually contained in the retroperitoneum can manifest with anemia and occasionally with common bile duct obstruction.

U/S of the abdomen: multiple gallstones and a slightly thickened gallbladder wall, normal hepatic bile duct diameter, and an AAA 4.7 cm in diameter. upper gastrointestinal endoscopy and colonoscopy, neither of which disclosed the cause of anemia. What would you do Next ?? CT scan of the abdomen showed Infrarenal aortic aneurysm that was 4.5 cm in diameter without evidence of rupture.

Bilateral small renal calculi, bilateral renal masses consistent with simple cysts were noted.
Day after admission .... Gross hematuria developed.

On further questioning, the patient reported a 2-month history of intermittent pinkish discoloration of urine without dysuria, frequency, or urgency.
Urine Analysis .. More than 100 erythrocytes
1 to 3 leukocytes per HPF
Results of urine Gram stain, cultures, and cytology were negative.
Urine eosinophils were 1 to 5% (normal, 0 to 5%).
A 24-hour urine protein level was 2,212 mg
Which one of the following is the most likely cause of hematuria in this patient? (Hematuria associated with Goodpasture's syndrome is usually preceded by hemoptysis. Our patient had no hemoptysis, respiratory complaints, or chest roentgenographic abnormalities) In our 68-year-old patient with asymptomatic hematuria, a urinary tract neoplasm as well as benign prostatic hypertrophy is the primary consideration.

a.Goodpasture's syndrome

b.Neoplasm of the urinary tract

c.Renal artery embolism

d.Glomerulonephritis

e.Genitourinary tuberculosis
Renal artery embolism must be considered in any patient with hematuria who has the classic risk factor of valvular heart disease or cardiac arrhythmia. A major noncardiac source of renal emboli is ruptured aortic atheromatous plaque. Although our patient had a prosthetic heart valve and was receiving oral anticoagulant therapy, the occurrence of renal artery embolism is not precluded Glomerulonephritis in patients older than 40 years rarely is associated with gross hematuria. The most common glomerular disease associated with asymptomatic gross hematuria is IgA nephropathy. Genitourinary findings seldom are an isolated manifestation of tuberculosis. Pulmonary disease is usually present, and the clinical features are subacute to chronic, rather than acute as in our patient. Usual associated findings are cystitis and sterile pyuria instead of hematuria What would yo do next depending on your previous choice ? On cystoscopy Enlarged prostate with bleeding vessels, which were fulgurated successfully.
Mild hematuria persisted despite fulguration, and the cause of his intermittent hematuria was unclear.
Which one of the following is least likely to have contributed to the renal insufficiency in our patient?
a.Atheroembolic disease of the renal artery

b.Renal malignant lesion

c.Glomerulonephritis

d.Hypertensive nephrosclerosis

e.Hydronephrosis They are frequently seen after manipulation of the vessels, such as for aortic surgical procedures or arteriography.
commonly involve other organs, such as the retina, skin (livedo reticularis), muscle, and brain.
Our patient had no clinical features of embolic involvement of other organs. Renal malignant lesion can cause renal failure by three mechanisms:
direct infiltration of the renal parenchyma,
Immune factors
postrenal obstruction due to direct compression by the tumor or enlarged lymph nodes.
In our patient, a CT scan of the abdomen revealed bilateral renal masses. With the presence of hematuria, a renal malignant tumor must be considered a potential cause of the renal failure. Because of the presence of hematuria and proteinuria Our patient had a 20-year history of hypertension that could contribute to the renal insufficiency,
it is unlikely to be the only cause of the severe decline in renal function from a creatinine level of 1.4 to 4.1 mg/dL during a period of 2 years. would be easily detected on ultrasound studies or CT scans of the abdomen.
In our patient,both ultrasonography and CT scans of the abdomen were negative for hydronephrosis. Further Evaluation ESR : 38 mm
RF : 243 IU/ml
ANCA -ve
Anti GBM -ve

Would You biopsy ? Renal Biopsy showed :
Evidence of IgA nephropathy and a small cell malignant lymphoma of B-cell origin.
Bone marrow aspiration also showed lymphomatous involvement. The oral coumarin therapy was discontinued, and intravenous administration of heparin was initiated before the renal biopsy was performed.
would you suggest any further investigations ? In the context of the histologic findings from the renal biopsy in our patient, which one of the following statements is true? a.A direct association exists between IgA nephropathy and chronic lymphocytic leukemia and B-cell lymphoma

b.IgA nephropathy is a benign disease

c.Serum total IgA is increased in 90% of adults with IgA nephropathy

d.Lymphomatous infiltration of the renal parenchyma is a common cause of renal failure in patients with lymphoma

e.Primary renal lymphoma is a rare occurrence Progression In our patient, coumarin therapy was resumed 3 days after the renal biopsy. Approximately 2 days later, he noted acute left flank pain. The prothrombim time and INR were 20.2 seconds and 2.1, respectively. An emergency abdominal CT scan revealed a large hematoma in the left perinephric area.

Our patient did well with conservative management of the perinephric hematoma.

he was given 2 units of packed erythrocytes. Anticoagulant therapy was withheld, and the hemodynamic status was closely monitored; however, the creatinine level increased from 3.5 to 4.7 mg/dL. Because of his poor renal function, we decided to treat his lymphoma with prednisone, 80 mg/day orally. At 2 months, renal function had improved, and the creatinine level was 2.0
In about 30% of patients with IgA nephropathy, end-stage renal failure develops within 20 years after diagnosis Serum total IgA is increased in 33 to 50% of adults with IgA nephropathy. Serial determinations have not been correlated with the severity or activity of the disease. Therefore, this determination is not useful in diagnosing or following the course of IgA nephropathy Lymphomatous infiltration of the kidneys rarely causes renal failure. Thank You :)
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