Case 1 - Click here for Text and References

Submitted by: Agnes B. Fogo

Clinical Summary:

A 42 year old Caucasian male with HIV and history of non-Hodgkin’s Lymphoma presented to this hospital with one week of malaise, weakness, and fever to 101. He stated that the symptoms had been progressive, and also noted darker and intermittently bloody urine for a few days without dysuria. He also reported lumbar paraspinal pain and colicky left lower quadrant discomfort. He reported exposure to a co-worker with a flu-like illness. Three days prior to presentation, he presented to clinic with complaints of fatigue, back soreness, and dark urine. Urinalysis at that time was significant only for 8 RBCs seen on microscopy.

His past medical history was significant for HIV, diagnosed in 2002, most recent CD4 count ~600s with undetectable viral load six months earlier. History was also significant for stage III non-Hodgkin’s Lymphoma (atypical Burkitt’s) diagnosed in 2004 that was in remission after MEGA III chemotherapy (cyclophosphamide, etoposide, vincristine, bleomycin, adriamycin, methotrexate/leucovorin, and prednisolone). Medications at the time of presentation included Atripla (efavirenz, emtricitabine, and tenofovir) and zolpidem. Family history included diabetes in his father, Alzheimer’s dementia in his mother, and cervical cancer in his sister. He was divorced and in a monogamous homosexual relationship for six years, quit smoking tobacco six years prior, drank alcohol occasionally, but denied illicit drugs. He worked as a construction estimator.

On examination, he was well-appearing and in no acute distress. His temperature was 100.7 F, HR 91, BP 124/78, and his oxygen saturation was 98% on room air. HEENT, respiratory, and cardiac exams were all normal. His abdomen was soft with mild tenderness to palpation in the left lower quadrant without guarding or rebound. He had no hepatosplenomegaly. He had no CVA tenderness, no midline tenderness, and no stepoff upon palpation of the spine. He had no penile or anal lesions. He had full range of motion and no edema or clubbing. His neurologic exam was non-focal. He had no rash or jaundice.

Initial laboratory evaluation showed WBC 7.2 (ANC 4.4), PCV 39 (MCV 93), and Plts 181. Metabolic profile showed sodium 139, potassium 4.2, chloride 105, bicarbonate 21, BUN 17, creatinine 1.9 (baseline 0.9), glucose 82, and calcium 9.3. Total protein was 8.3, albumin was 5.0, total bilirubin 0.5, alkaline phosphatase 100, AST 20, and ALT 29. Creatine kinase was 36 and haptoglobin was 317 (nl 16-200). INR was 1.2, and PTT 38.7 (control 29.0). RPR was negative. Urinalysis showed specific gravity 1.006, protein 30, negative nitrite and leukocyte esterase, large blood, and otherwise negative. Urine microscopy showed 1 WBC, 542 RBCs, and no eosinophils. There were no casts but presence of dysmorphic RBCs was noted. A single spot urine evaluation showed sodium was 56, creatinine was 0.46, and protein was 0.41. Fractional excretion of sodium was calculated at 1.66%.

Chest x-ray showed mild atelectasis. Lumbar x-ray showed only L5 spondylolysis. Renal ultrasound showed R kidney 12 cm and left kidney 11.8cm in length. No hydronephrosis, calculi, or cystic masses were seen. Mild increased renal cortical echogenicity was seen. Magnetic resonance venogram did not show renal vein thrombosis and showed minimal cortical scarring.

Further lab results included anti-streptolysin O antibody of 177 (equivocal) with repeat value of 159 two days later, C3 was 152 (nl 88-201), and C4 was 31 (nl 15-45). Erythrocyte sedimentation rate was 84, and c-reactive protein was 233. Blood and urine cultures remained negative throughout his hospitalization.

Despite fluid hydration and cessation of possible offending drugs, his creatinine continued to rise to 4.0 within his first six days in the hospital. His urine output was fairly stable between one and three liters per day. He had intermittent fevers to 101 for the first five days of his hospitalization then was afebrile thereafter.

A diagnostic procedure was performed.

Case 2 - Click here for Text and References

Submitted by: Vivette D. D'Agati

Clinical Summary:

A 26-year-old Caucasian female was found to have asymptomatic renal insufficiency on routine laboratory studies. She denied history of gross hematuria, edema or oliguria. She had no history of diabetes, hypertension, arthritis, urinary tract infection or nephrolithiasis. Her only medication was an oral contraceptive (Lo/Ovral). On physical examination, she had BP 120/66 mm Hg, heart rate of 70 per min and weight of 146 lb. Physical examination was unremarkable, with no evidence of rash or edema. Laboratory examination showed a total leukocyte count of 7000/mm2, Hct 39.8%, platelet count 242 x109/L, 24 hour urine protein 70 mg, serum albumin 4.3 g/dL, serum creatinine 1.6 mg/dL, creatinine clearance 71 ml/min, serum cholesterol 222 mg/dL. Serum electrolytes were normal including sodium, potassium, glucose, bicarbonate and calcium. The following serologies were negative or normal: ANA, anti-dsDNA Ab, ANCA, HBsAg, HCV antibody, C3 and C4. Urinalysis showed specific gravity 1.010, pH 5.0, and was negative for protein, blood, glucose and nitrite. Microscopic examination of the urine revealed 0-5 WBC and no RBC per high power field, and no casts. Renal ultrasound showed normal-sized kidneys with normal echogenicity and no hydronephrosis, masses or cysts. A renal biopsy was performed.

Pertinent Laboratory Data:

Light microscopic and electron microscopic images are provided. By immunofluorescence (not shown), there was segmental 1+ mesangial staining for IgM and C3 only in the 2 glomeruli sampled. Tubular casts stained 1+ for IgA, kappa light chain and lambda light chain.

Case 3 - Click here for Text and References

Submitted by: Arthur H. Cohen

Clinical Summary:

Sixty-nine year old woman with type 1 diabetes mellitus since age 8 with ultimately developing end-stage diabetic nephropathy received a deceased donor renal transplant in 1991. She did well, maintained on cyclosporine and prednisone, until 2010 at which time serum creatinine was noted to be in the range of 2.0 mg/dl. Biopsy in 2010 disclosed changes consistent with chronic calcineurin- inhibitor toxicity.

In February, 2012 she developed a foot infection. This was ultimately determined to be caused by methicillin-resistant staph aureus and was treated with vancomycin. At about that time she underwent surgical resection of portion of bone. She was hospitalized in beginning of May 2012 with diabetic ketoacidosis, sepsis, hypotension and acute oliguric renal failure with serum creatinine 5.0 mg/dl. She was treated with fluids and restarted on vancomycin as wound culture grew methicillin-resistant staph aureus. Serum creatinine improved to 1.5 mg/dl. Two weeks later, outpatient visit disclosed serum creatinine of 3.7 and she had low urine output. Urinalysis indicated specific gravity 1.009, pH 5.0, protein 2+, leukocyte esterase 3+, blood 3+ and numerous red blood cells and white blood cells. Leukocytes were identified as 96% neutrophils, 1% lymphocytes, and 3% eosinophils. Urine culture was negative and urine protein/creatinine ratio was 2.3. Cyclosporine and vancomycin were continued. Hemodialysis was stated on May 26 and biopsy was done a few days later. Additional laboratory evaluation included negative serologies for hepatitis B and C.

Case 4 - Click here for Text and References

Submitted by: Robert B. Colvin

Clinical Summary:

A 39 year old woman was seen for progressive edema.

She has a history of systemic lupus erythematosus, symptomatic at age 10 years and diagnosed 6 years later by a rheumatologist in Brooklyn, manifested by a butterfly rash, pleurisy, arthralgias and severe Raynaud's phenomenon. A renal biopsy then showed "mild kidney disease." She was treated with Plaquenil and prednisone and has had no recent flares. She had been on no medical care or medicines > 5 years due to lack of insurance. No family history of SLE or scleroderma; mother and grandmother have rheumatoid arthritis.

Six months ago she came to the Emergency Department for recent onset of "flu" with a non-productive cough and shortness of breath, which responded to antibiotics. Imaging studies detected chronic lung disease with honeycombing in both lung bases, most consistent with mixed connective tissue disease. In addition a patulous esophagus was detected. Laboratory studies at that time workup revealed ANA+ 1:640 speckled, with positive Ro and RNP; negative LA, dsDNA, SM, scl70, ANCA‎. Negative antiphospholipid antibodies and normal complement (C4, C3).‎ Cr 1.3 mg/dl after hydration. Urinalysis: 2+ protein, no rbc casts. Overall the clinical impression was MCTD/SLE with scleroderma features. She made an uneventful recovery and was started on Plaquenil.

About a month ago she noticed that her legs were getting swollen and about 7-10 days ago the swelling went up to her thighs and abdomen, gaining 30 lbs. PE: Normal vital signs. Sclerodactly and periungal erythema on her fingers bilaterally. Some tightening of skin over her forarms. No tenderness,swelling erythema of her small or large joints in upper and lower exterimities.‎ Labs: Serum albumin 1.5 gm/dl, Cr 0.7 mg/dl; urine protein/Cr 8.4 (19.8 gm/24 hrs), Cr 07 mg/dl. Urine: 3+ protein, 5-10 RBC, no RBC casts. ANA 1:160 (speckled), dsDNA 1:40; normal C4, C3.

She was treated with Plaquenil, Cellcept and prednisone. Two months later her urine protein decreased to 3.4. When last seen 3 years after the renal biopsy, she had no further pulmonary symptoms or edema, her urine P/Cr ratio was 0.7, serum albumin 4.2 gm/dl, anti-RNP positive (161 EU/ml, nl 0-20), anti-ds DNA negative, C3, C4, normal,

Case 5 - Click here for Text and References

Submitted by: Christopher Larsen

Clinical Summary:

The patient is an 81 Caucasian male hospitalized for treatment of cellulitis and found to have worsening renal function and proteinuria.

Past medical history included diabetes mellitus, hypertension, and osteoarthritis with occasional NSAID use. The patient’s medications included vancomycin, Zosyn, lisinopril, and glipizide.

On physical exam the patient was afebrile and hypertensive with a blood pressure of 142/82. There was 3+ LE edema and slight obesity. The exam was otherwise unremarkable without a rash.

Pertinent Laboratory Data:

His serum Cr was increased at 2.6 mg/dL (baseline 1.5 mg/dL). Urinalysis showed 2+ blood, 3+ protein, negative glucose, and normal pH. Twenty-four hour urine collection revealed 6.7 grams of proteinuria/day. He had an elevated sedimentation rate a 73 (normal <13) and a positive cANCA with at titer of 1:80. ANA was negative. His free light chain assay was abnormal with a kappa/lambda ratio of 0.03. C3 and C4 were within normal limits at 114 mg/dL and 14 mg/dL, respectively. Electrolytes revealed mild hypokalemia with a potassium of 3.3mmol/L (normal 3.8-5.0). Ca, Cl, Mg, Na were all within normal limits. CBC was notable for mild anemia with Hgb 12.1 G/dL (normal 13-17).