Case time! I've got a good one for PAs and students alike

[Joelseff]

humm bladder obstruction leading to hydro?? sort of rules out CRI ..... I am out of ideas too - - - as a renal doc once told me "you just have to listen to what the kidney is telling you " but I think I am kidney deaf....

 

Enlarged kidneys are expected in HIVAN, but pts cd4s are high...hmmmm could be iatrogenic from the reyataz, may rebound since reducing dose but??? let's wait for nephro recs

 

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[VictoriaO]

Ok, I know this trail may be getting a little cold, but if there are any ID or nephro nerds out there (raises hand), this gets pretty interesting.

Over the weekend the pt's Cr continues to rise 7.18 to 7.77 to 8.08 to 8.31 despite hydration and decrease in her atazanavir dose (we held her pravastatin to r/o rhabdo though her CK was normal). Pt now stating her face "feels puffy", she has had frontal headaches and nausea (possibly related to uremia), her head CT is reviewed again. On Sunday she spikes a temp overnight to 38.2, comes down with tylenol. Her sinuses are scanned, CT is below. Chest films are taken. Urology consults and gets a non-con abdominal CT seen below, they then sign-off and will do a cystoscopy in clinic, but they find it unlikely the hematuria is unrelated to her renal failure. Pt still making copious pink urine.

 

HISTORY: Sinus pain, frontal headache. TECHNIQUE: Images in the axial plane are obtained through the sinuses without contrast and reformatted in the sagittal and coronal plane as per landmark protocol. COMPARISON: No prior FINDINGS: Mild opacification seen of the right frontal sinus along with presence of frothy secretions. Opacification noted in the right frontoethmoidal recess.. Mild mucosal thickening is identified in bilateral maxillary sinuses. Rest of the paranasal sinuses are well-aerated. The visualized portions of the orbits are unremarkable. Limited visualization of pain shows hyperdensity in the right globus pallidus likely secondary to mineralization. Hypodensities noted in the periventricular white matter. IMPRESSION: Opacification of the right frontal sinus and frontal ethmoidal recess with frothy secretions suggestive of sinusitis. Minimal mucosal thickening in the maxillary sinuses.

 

CT ABDOMEN AND PELVIS without contrast 3/31/2012 COMPARISON: Ultrasound kidney and bladder 3/30/2012 CLINICAL DATA: Acute kidney injury. TECHNIQUE: Overlapping 5 mm images were obtained through the abdomen and pelvis without contrast. FINDINGS: Abdomen: Imaged lung bases are clear. Lack of intravenous contrast limits evaluation. Unenhanced liver demonstrates a segment 6 subcentimeter hypodense lesion, which is too small to accurately characterize, statistically likely a cyst. Gallbladder is contracted. Spleen is normal. Mild pelvocaliectasis seen on yesterday's ultrasound involving the left kidney is not appreciated on this CT. no renal, ureteral or bladder calculi. No hydronephrosis. Adrenal glands, pancreas, stomach, small and large bowel and appendix are normal. No intraperitoneal free fluid. Non-aneurysmal aorta demonstrates moderate atherosclerotic calcifications. Pelvis: Rectosigmoid colon and bladder are normal. No gross abnormality of the uterus or adnexae. Small amount of simple appearing free fluid is present in the pelvis. Musculoskeletal: No suspicious osseous lesion. IMPRESSION: Small amount of simple appearing free fluid in the pelvis, of uncertain etiology. Otherwise unremarkable noncontrast CT of the abdomen/pelvis.

 

 

EXAM: CHEST 2 VIEWS 04/01/12 10:53:00

 

HISTORY: Cough.

 

COMPARISON: None.

 

FINDINGS: The cardiomediastinal silhouette is normal in size and

contour. There is mild bibasilar atelectasis. Right middle lobe

opacity is thought to represent confluence of vessels and bronchi.

There is no focal consolidation or suspicious pulmonary nodule.

There is no pleural effusion or pneumothorax. Mild degenerative

changes are noted in the thoracic spine. The osseous structures are

intact.

 

IMPRESSION:

 

Mild bibasilar atelectasis. Otherwise no acute intrathoracic

abnormality.

Urine is spun again, still showing very minimal brown casts and gross monomorphic reds.

Complements are back and they are normal

SPEP is back, low albumin but otherwise normal. ASO titer is negative.

The following is still pending: HIV viral load, CD4, Hep B, C, cryptococcal antigen, urine cytology, ANCA, anti-GBM, (and a few others, I'll post more tomorrow when I'm back)

 

Would a white female get HIV nephropathy? Would it cause such acute renal failure? What else could cause diffusely enlarged echogenic kidneys?

What else is still on the d/dx, there are some intra-renal pathologies (AGN, ATN, AIN) and some other zebras that need to be ruled out (normal complements are helpful) One thing we thought, did the protienuria start first or did the hematuria? Can you chalk up all of her headache and nausea to uremia?

[RRivas2004]

This is a ddx list I found that seems pertinent to the case, I copied and pasted from this website, so all credit belongs to the author... ( http://roentgenrayreader.blogspot.com/2010/08/bilateral-enlarged-kidneys-differential.html )

 

[h=3]Bilateral Enlarged Kidneys: Differential Diagnosis[/h]

Here, we'll consider bilateral enlarged kidneys without focal masses.

• Diabetic nephropathy: Most common cause of bilateral enlarged kidneys, accounting for 50% of cases. May precede chemical evidence of diabetes by a year or so. The echogenicity of the kidneys is normal.
  
• Acute glomerulonephritis: Enlargement is thought to be due to edema. Patients present with renal insufficiency.
  
• Vasculitis/Autoimmune: Systemic lupus erythematosus, Wegener (sinusitis and hemoptysis), Goodpasture (pulmonary hemorrhage may be present), Henoch-Shonlein (diffuse purpura), polyarteritis nodosa (multiple aneurysms may be seen on angiography).
  
• HIV nephropathy: Enlarged, echogenic kidneys with decreased corticomedullary definition, decreased renal sinus fat, parenchymal heterogeneity, and globular renal configuration. About 10% will have medullary hyperattenuation. Striated nephrograms may also be seen.
  
• Leukemia/Lymphoma: Diffuse infiltration of the kidney with preservation of renal architecture and contour and decreased echogenicity seen early. Later, there is an expansile heterogeneous renal mass with loss of normal renal architecture.
  
• Autosomal recessive polycystic kidney disease: Massively enlarged hyperechoic kidneys with loss of corticomedullary differentiation.
  
• Acute interstitial nephritis: Enlarged echogenic kidneys. Initially reported in association with scarlet fever, acute interstitial nephritis is also known as hypersensitivity nephritis and can be due to humoral and cell-mediated responses to infection or medication.
  

[Joelseff]

Ok, I know this trail may be getting a little cold, but if there are any ID or nephro nerds out there (raises hand), this gets pretty interesting.

Over the weekend the pt's Cr continues to rise 7.18 to 7.77 to 8.08 to 8.31 despite hydration and decrease in her atazanavir dose (we held her pravastatin to r/o rhabdo though her CK was normal). Pt now stating her face "feels puffy", she has had frontal headaches and nausea (possibly related to uremia), her head CT is reviewed again. On Sunday she spikes a temp overnight to 38.2, comes down with tylenol. Her sinuses are scanned, CT is below. Chest films are taken. Urology consults and gets a non-con abdominal CT seen below, they then sign-off and will do a cystoscopy in clinic, but they find it unlikely the hematuria is unrelated to her renal failure. Pt still making copious pink urine.

 

HISTORY: Sinus pain, frontal headache. TECHNIQUE: Images in the axial plane are obtained through the sinuses without contrast and reformatted in the sagittal and coronal plane as per landmark protocol. COMPARISON: No prior FINDINGS: Mild opacification seen of the right frontal sinus along with presence of frothy secretions. Opacification noted in the right frontoethmoidal recess.. Mild mucosal thickening is identified in bilateral maxillary sinuses. Rest of the paranasal sinuses are well-aerated. The visualized portions of the orbits are unremarkable. Limited visualization of pain shows hyperdensity in the right globus pallidus likely secondary to mineralization. Hypodensities noted in the periventricular white matter. IMPRESSION: Opacification of the right frontal sinus and frontal ethmoidal recess with frothy secretions suggestive of sinusitis. Minimal mucosal thickening in the maxillary sinuses.

 

CT ABDOMEN AND PELVIS without contrast 3/31/2012 COMPARISON: Ultrasound kidney and bladder 3/30/2012 CLINICAL DATA: Acute kidney injury. TECHNIQUE: Overlapping 5 mm images were obtained through the abdomen and pelvis without contrast. FINDINGS: Abdomen: Imaged lung bases are clear. Lack of intravenous contrast limits evaluation. Unenhanced liver demonstrates a segment 6 subcentimeter hypodense lesion, which is too small to accurately characterize, statistically likely a cyst. Gallbladder is contracted. Spleen is normal. Mild pelvocaliectasis seen on yesterday's ultrasound involving the left kidney is not appreciated on this CT. no renal, ureteral or bladder calculi. No hydronephrosis. Adrenal glands, pancreas, stomach, small and large bowel and appendix are normal. No intraperitoneal free fluid. Non-aneurysmal aorta demonstrates moderate atherosclerotic calcifications. Pelvis: Rectosigmoid colon and bladder are normal. No gross abnormality of the uterus or adnexae. Small amount of simple appearing free fluid is present in the pelvis. Musculoskeletal: No suspicious osseous lesion. IMPRESSION: Small amount of simple appearing free fluid in the pelvis, of uncertain etiology. Otherwise unremarkable noncontrast CT of the abdomen/pelvis.

 

 

EXAM: CHEST 2 VIEWS 04/01/12 10:53:00

 

HISTORY: Cough.

 

COMPARISON: None.

 

FINDINGS: The cardiomediastinal silhouette is normal in size and

contour. There is mild bibasilar atelectasis. Right middle lobe

opacity is thought to represent confluence of vessels and bronchi.

There is no focal consolidation or suspicious pulmonary nodule.

There is no pleural effusion or pneumothorax. Mild degenerative

changes are noted in the thoracic spine. The osseous structures are

intact.

 

IMPRESSION:

 

Mild bibasilar atelectasis. Otherwise no acute intrathoracic

abnormality.

Urine is spun again, still showing very minimal brown casts and gross monomorphic reds.

Complements are back and they are normal

SPEP is back, low albumin but otherwise normal. ASO titer is negative.

The following is still pending: HIV viral load, CD4, Hep B, C, cryptococcal antigen, urine cytology, ANCA, anti-GBM, (and a few others, I'll post more tomorrow when I'm back)

 

Would a white female get HIV nephropathy? Would it cause such acute renal failure? What else could cause diffusely enlarged echogenic kidneys?

What else is still on the d/dx, there are some intra-renal pathologies (AGN, ATN, AIN) and some other zebras that need to be ruled out (normal complements are helpful) One thing we thought, did the protienuria start first or did the hematuria? Can you chalk up all of her headache and nausea to uremia?

 

HIVAN is statistically higher in Afro americans but very possible and actually her high cd4 does not exclude it.

 

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[RRivas2004]

Need biopsy to R/O Wegners, The patient has symptoms of a small vessel vasculitis, I keep circling around back to wegners because of her sinusitis, cough, and the kidney injury. A trial of corticosteroid therapy might show some positive results? and could lead us more in the direction of vasculitis.

[Joelseff]

My thoughts are surrounding HIVAN which has an auto immune component, no one is sure but has been suggested in HIV circles. I would consider starting an ACEi and definitely get a bx.

[sk732]

Only other thing I can think of that I might be able to toss in is IgA nephritis, given feeling "fluish" prior and the painless hematuria.

 

Any foreign or domestic travel?

 

SK

[VictoriaO]

So the verdict is in!

 

(SK, no foreign or domestic travel).

 

Here's how the case plays out. Cr continues to rise, 8-9-10, pt gets dialyzed. On the day before dialysis she becomes pancytopenic with a CBC looking like this 2.3/9.3/23/137. Afebrile, still making great urine. Here CBC bounces back to 3.5/9.6/24/140. We needed to rule-out bilateral renal vein thrombosis prior to the biopsy (she didn't have dopplers on her initial ultrasound, that's what I was getting at with protienuria coming before hematuria). Here are the labs:

 

Anti-GM: IgG +, IgA -

ANCA -

CD4 175

HIV-PCR undetected

ASO -

Hep C Ab -

Hep B SAg -, SAb -

RPR non-reactive

Influenza A/B not detected

Respiratory viral panel neg

Cryptococcal Ag neg

SPEP low total protein

Pending:

Anti-GM Elisa

Renal bx:

 

So this ended up being a very interesting discussion between myself, ID and Nephro about whether to start IV methylpred. Apparently 17% of HIV pts have positive anti-GBM antibodies without renal disease, this is typically associated with lower CD4 counts. There are some case reports of these patients ending up having IgA nephropathy on biopsy or other etiologies. Our nephrologist didn't think the timeline fit with HIV nephropathy (he's never heard of a case in a white female), Goodpasture's is a fairly rare disease, and there still may be an infectious etiology. Our d/dx also included BK virus (mainly affects renal transplant pts, but can cause hematuria and renal failure in HIV patients but is difficult to test for), adenovirus (can cause hemorrhagic cystitis and AIN in HIV pts), though a viral illness could have been the stimulus for the production of anti-GBM. We decided to start IV methylpred, but hold off on the plasmapheresis.

 

I'll post as soon as I know what the biopsy shows

 

http://www.medscape.com/viewarticle/572796_3

http://www.nature.com/nrneph/journal/v4/n3/full/ncpneph0724.html

[Mastersoldier13]

Im just a PA student but by the looks of all the pt sx and finding, its deffinetly a systemic cause. Im thinking its SLE(Lupus). Although renal and CNS sx are not as common, they exist. And it fits with the arthralgias, maybe even the rash she got and first degree heart block since it affects the heart, the N/V could also be explained by it. This disease has fluctuating courses which can explain her past sx which might have gone into remmision before. And although its not too pertinet, flares of lupus have been associated with a post ovulatory state, and they tend to go into remmision with the onset of menses. Since she spiked a fever and you tx with tynelol and got a good response that also is a good sign. On the CXR the consolidation might have been from a pulmonary infiltrate. Well thas my 2 cents, maybe if we get the results of the ANA we'll see what to do from there. Btw im not trying to have tunnel vision here, just shouting out what i think is most likely. Everyone elses comments are just as good if not better than mine and i would deffinetly include in my ddx.

[RRivas2004]

Sweet, I was close! :=-0: I'm sure the renal bx will shed some more light once the pathologist takes a look at it. I didn't even know about Anti-Gm great case.

[Mastersoldier13]

Wow Ig G nephropathy, they were briefly mentioned that i would have just said Ig A. Subtypes are really challenging thing to dx since exposure to them is lessened. Im gona begin focusing on finding the variants of dz. Dont want to get confused when i find things that dont match up. Thanks for the case, it really helps take something away from which we learn from.

[VictoriaO]

And the biopsy shows.......It's Goodpastures! Definitely rapidly-progressing (Cresenteric) glomerulonephritis. Yes, very rare, one in a 1 million case. The formation of anti-GBM can be triggered by a viral infection or by inhalation of chemicals (pt told me today she works next to a nail salon and the fumes bother her). Interestingly smokers are more prone to lung manifestations of Goodpastures, luckily she does not have these. Interestingly nephrology does not seem to think she at risk for re-presenting with the lung manifestations. What I would be very interested to read is if this 17% of HIV pts with circulating anti-GBM is susceptible to some sort of trigger for this to start depositing in the kidneys and causing renal failure. Very interesting (and unlucky!) that this woman would have Goodpastures (a 1 in a million or 2 million disease in whites) and HIV.

 

Right now she's getting set up for ongoing dialysis, she may be a transplant candidate, she's pretty optimistic about keeping her life as normal as possible while out dialysis. I came in there this morning and she was reading a huge packet of info about renal diet. I'm hopefull she'll do as well as she can.

 

 

I'll try and do another case soon if people are interested. Right now we've got a capillary leak syndrome case (who just went to the unit), and a pancytopenia with platelets of 2 and tons of petechiae all over without prior medical hx. Plus all the other bread-and-butter hospitalist stuff.

[RRivas2004]

What threw me off in this case was that Wegners had more of the upper respiratory symptoms like the sinusitis on CT as compared to Goodpastures which typically does not... I was pointing my finger at her chronic sinusitis as the trigger for the renal injury.

[Piper]

"I'll try and do another case soon if people are interested. Right now we've got a capillary leak syndrome case (who just went to the unit), and a pancytopenia with platelets of 2 and tons of petechiae all over without prior medical hx. Plus all the other bread-and-butter hospitalist stuff."

 

YES, please! That was a great case study. Keep 'em coming when you can; great workout for my brain which is currently drowning in runny noses at urgent care. Nice job - I wish the best for your patient.

[VictoriaO]

My apologies for the poor sentence structure in my last post, upon re-reading it I realized I used the word "interesting' about 5 times. No more posting without coffee for me.

 

I think the patient will end up doing well, she's very motivated to get back on her feet. She's been a real joy to work with, she kept laughing that we couldn't figure out what was wrong. Now she's reading everything she can get her hands on about renal diet and maximizing her dialysis. I can only imagine what type of luck she must have to get possibly lyme disease, HIV, encephalopathy (PML vs AIDS which she recovered from) and now Goodpasture's. I'm just glad it wasn't something like BK virus causing renal failure and hemorrhagic cystitis which would have been a nightmare to treat. Interesting as well that she didn't present with hypertension, I'm wondering if her ARB and BB could have presented that to some degree.

[jmj11]

It was a very interesting and educating case. It reminds me what a steep learning cure I would need to be a hospitalist. I even worked (for just a year) with a combination nephrologist /rheumatologist's practice. But that was so long ago that we were still testing urine by sipping it. :>)

[deborah212]

Good case.

[crystaltide]

I'll try and do another case soon if people are interested. Right now we've got a capillary leak syndrome case (who just went to the unit), and a pancytopenia with platelets of 2 and tons of petechiae all over without prior medical hx. Plus all the other bread-and-butter hospitalist stuff.

 

I am interested! This case was great as we just finished covering our renal unit in pathophys. Thanks!