Sunday, October 09, 2005

Effusion, ascites, and liver nodules, Oh my!

Here's a post from a guest:

55 yo female from Africa developed pleuritic chest pain, dyspnea, and LE in early fall of the prior year. She was initally treated with ACE-I and Lasix. Her symptoms worsened. With additional water restriction she would respond but suffered repeated bouts and progressive worsening of the symptoms each time.

She was admitted to an OSH with the above symptoms when visiting her daughter in the U.S. in July of this year. Ten pound weight loss noted over the last 6 months. Additionally, she had N/V, abdominal distension, and decreased urine output. Pertinent labs: Bun/Cr 240/18, K 7.6, HCO 18.
On exam, significant edema with some skin breakdown and ulceration secondary to edema.
CXR w/ large R. pl effusions. Abd U/S w/ small kidneys. Abd CT with diffusely nodular liver, EKG showed sinus bradycarda, low voltage QRS, small lateral Q waves interpreted as possible infarct.
She was started on HD. R. Pl effusion was sampled and c/w exudative pl effusion, negative smears, PCR for MTB negative, ADA negative. Paracentecis negative cytology for malignancy.

U/S guided liver Bx X2 performed demonstrated granulomas and possible intracytoplasmic organisms.

Pt. developed respiratory compromise and was intubated after bradycardic arrest on the way to OR for VATS Bx. Pt. developed Serratia PNA. She was transferred to the MICU at our institution.

PMHx: syphyllis, malaria, HTN
Social: non smoker

Radiographs from our institution:





What do your differential; any other tests?

Addendum: Nice differential CEOB. Here's some more information with a representative picture of histo consistent with both liver Bx and transbronchial bx:



Path and Diagnosis:
TBBX: Amyloid
Liver Bx: Serum Amyloid A protein
Pleural flulid Culture: MTb

In case you missed the last update - this is yet another example of TB being sneaky. However, MTb should always be considered with any infectious presentation from abroad. In this patient, chronic infection lead to deposition of serum amyloid A protein (SAA) in more than one organ. SAA is an acute phase reactant that can be seen in chronic inflammatory conditions such as rheumatoid arthritis, chronic infection, and Familial Mediterranean Fever.

17 comments - CLICK HERE to read & add your own!:

Jeff H said...

I saw this patient, so will not be commenting on this case.

Arenberg said...

"I saw this patient, so will not be commenting on this case."

I don't mean to nitpick, but technically speaking, didn't you just comment?

OK...maybe I did mean to nitpick

Baleeiro said...

Where is she from in Africa?

Jeff H said...

Hey Doug, how's about you quit picking on me and actually discuss your thoughts on the case????

Baleeiro said...

There are quite a few problems going on here (duh!). She has granulomatous liver disease, coming from Africa I would be concerned about Schistosomiasis (the granulomata form around the eggs). There are different species of Schistosoma and all the clinically relvant ones (except for mekongii and japonicum) are endemic in Africa. Mansonii and intercalatum cause liver disease and may actually lead to pulmonary hypertension as the eggs gain access to pulmonary circulation. The right effusion is likely a hepatic hydrothorax secondary to the portal HTN. Interestingly, usually liver synthetic function is preserved in peri-portal fibrosis unlike in cirrhosis.

Baleeiro said...

Now, regarding the renal failure, mansonii and intercalatum cause GI and liver disease (as well as Pulm HTN) but not renal failure. I will suggest two possibilities: Schistosoma hematobium causes egg deposition in the bladder. It may cause ureteral obstruction leading to renal failure because of the granulomata and rarely it leads to squamous cell Ca of the bladder (not transitional cell) which may also lead to obstructive uropathy.
Now the description of the kidneys and the anasarca seemed more like advance intrinsic renal failure with some nephrotic syndrome (did she have a lot of protein in the urine?). She has had malaria before. Chronic or recurrent malaria leads to a membranous GN and nephrotic syndrome. Acute malaria (usually falciparum) can lead to a severe presentation ("black water" fever) with pigment-induced nephropathy (all the hemoglobin from hemolyis).

Baleeiro said...

The right hilum seems a bit full as well. Malaria and some of the chronic diseases I mentioned may lead to secondary amyloid and that might be what I am seeing in the hilum. Did she have a lot of air space disease before the Serratia pneumonia?

Arenberg said...

It just occurred to me that this lady was admitted to my service the last day of my ICU rotation a few weeks ago. I know the answer and I still can't come up with a reasonable differential diagnosis.

How's that Jeff?

Mendez said...

She's from Zambia.

Mendez said...

About the ASD - OSH CXRs showed large effusion - there may have been ASD vs compressive atelectasis. I'll wait to see if there are anymore comments and post more info in a little while.

Baleeiro said...

Most of Sub-Saharan Africa (such as Zambia) is an endemic area for the infections I had mentioned. HIV and TB are also highly prevalent in Zambia and I hope an Hiv test was done at some point. There are also several Rickettsial diseases that are endemic in Central Africa (in light of those intracellular organisms).
Kala-azar (visceral leishmaniasis) will cause bone marrow suppression, immunocompromise, hepatosplenomegaly and may account for the intracellular organisms (quite typical on H&E) though I don't remember a direct renal involvement. This patients are usually hypogammaglobulinemic and GNR infections/sepsis are very common.

Baleeiro said...

I re-read the part where she has had syphillis before. With the high prevalence of HIV in Zambia I will suggest a co-infection with HIV and Kala-azar with HIV nephropathy and hepatic hydrothorax.

Baleeiro said...

This will be my last comment (I think). I will mention meliodosis since this is a pulmonary blog though I doubt it. It can occur sporadically in Central Africa though I'd expect more air-space disease to begin with. It can also affect liver, spleen and kidneys with focal abcesses though that is not the description we got...

Jeff H said...

Carlos, sometimes your knowledge base is downright frightening. Like Doug, I saw this patient and I know the diagnosis, and I'm still feeling like an intellectual peanut!

So, given the vast differential, how would you proceed to obtain a definitive diagnosis?

Baleeiro said...

Well, the path on the liver Bx would be great! I would get an HIV test, stool O&P and serologies for schistosoma, HIV and Kala-azar. When I was a kid we used to do skin testing for leishmaniasis (Montenegro reaction) but that could be negative in a hypoalbuminemic, critically ill Pt. The gold standard for Kala-azar and some of these weird tropical Rickettsia is a bone marro biopsy (with a spleen Bx having great sensitivity for Kala-azar).

Jennings said...

After carlos' 6 page answer, my addition to this case is "uh, yeah what he said".

Mendez said...

In case you missed the last update - this is yet another example of TB being sneaky. However, this Dx should always be considered with any infectious presentation from abroad.
In this patient, chronic infection lead to deposition of serum amyloid A protein (SAA) in more than one organ. SAA is an acute phase reactant that can be seen in chronic inflammatory conditions such as rheumatoid arthritis, chronic infection, and Familial Mediterranean Fever.