Alarming Presentation of a Seemingly Common Condition in a Patient with Renal Impairment

doi:10.4236/ojneph.2013.34031

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Open Journal of Nephrology, 2013, 3, 181-183

Published Online December 2013 (http://www.scirp.org/journal/ojneph)

http://dx.doi.org/10.4236/ojneph.2013.34031

Open Access OJNeph

Alarming Presentation of a Seemingly Common

Condition in a Patient with Renal Impairment

Mohammad Budruddin1*, Muhammad Yasir Khalil2, Issa Al Salmi2, Ramaiah Shilpa2

1Nephrology Department, Tawam Hospital, Al Ain, UAE

2Nephrology Department, Royal Hospital, Muscat, Oman

Email: *[email protected]

Received August 8, 2013; revised September 9, 2013; accepted October 2, 2013

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

We received a 23-year-old male, working in the Army for 18 months, with advanced renal impairment, haemoptysis

and hyperkalemia. An impression of the pulmonary renal syndrome was made and he was managed aggressively with

haemodialysis, plasma exchange and pulsing with methyl p rednisolon e. His condition improv ed, but the rena l functions

did not. The vasculitic workup including Extractable Nuclear Antigen (ENA) and Anti-Neutrophil Cytoplasmic Anti-

body (ANCA) which were sent on admission came out to be negative. As he apparently responded to the initial man-

agement protocol, he was subjected to renal biop sy which did not reveal any vasculitis but was consisten t for end-stage

renal disease. Subsequently he was subjected to lung biopsy which also was not supportive for vasculitis. However, we

concluded that the initial pointers for Wegener’s granulomatosis were misleading. The haemoptysis entirely subs ided as

the congestion improved. He was maintained on Haemodialysis three times per week schedule and discharged to the

peripheral health care facility.

Keywords: Haemoptysis; Renal Impairment; Pulmonary Renal Syndrome; Haemodialysis; ENA; ANCA; Vasculitis;

Pulmonary Oedema

1. Introduction

Pulmonary oedema is not an unusual presentation in pa-

tients with end-stage renal disease. This may be associ-

ated with haemoptysis. Radiological imaging of the chest

may at times be very confusing, mimicking alveolar

haemorrhage. We had a young patient who presented

acute onset advanced renal failure with haemoptysis. In

the course of his management, we concluded that his

haemoptysis was secondary to pulmonary oedema and

not pulmonary renal syndrome .

2. Case Summary

Mr. IRS, a 23-year-old Omani male was referred from a

peripheral Hospital with advanced renal failure and

haemoptysis. He was not know to have any medical pro-

blems in the past and was not on any medications before

this presentation. Neither was he ever told in any health

care facility to be having renal impairment. He presented

with the advanced renal failure with serum creatinine

more than 2000 mmol/l and urea 84 mmol/l. He was in

severe respiratory distress (tachypnoeic RR of 35/m, de-

saturating to 78% on room air) and developed massive

frank haemoptysis immediately after admission. The

chest X-ray Figure 1 on admission and urgent high res-

olution CT (HRCT) Figure 2 done after two sessions of

Haemodialysis with good ultrafiltration showed exten-

sive alveolar haemorrhage with perivascular nodules.

This concurred with our initial impression of pulmonary

renal syndrome (Wegener’s granolomatosis). Further CT

of the sinuses did show the polypoidal thickening of the

mucous membrane of the right frontal sinus. The com-

plements level done were normal.

His condition was critical with severe drop in Hb to 4

gm/dl. He was started on emergent aggressive treatment

for Wegner’s granulomatosis with 7 sessions of plasma

exchange and methylprednisolone 1 gm pulse daily for

three days. His haemoptysis subsided after the forth ses-

sion of plasma exchange and his general condition also

improved with improvement in the pulmonary conges-

tion and uremia following several sessions of haemodi-

alysis and ultrafiltrations.

*Corresponding author.

M. BUDRUDDIN ET AL.

182

The vasculitic workup subsequently came out to be

negative for Extactable Neuclear antigens (ENA), Anti-

Neutrophil Cytoplasmic Antibody but equivocal for An-

ti-glomerular basement membrane antibody (Anti GBM).

Serology for HBV, HCV and HIV were negative. Repeat

radiology showed improvement in the alveolar shadows.

Ultrasound estimation of kidney size was normal with

increased ecogenicity. The urine analysis was bland (no

active sediments) showed <2 RBC, <2 WBC, of 25 mg/L

Protien.

After stabilizing he was subjected to renal biopsy in

order to get the tissue diagnosis. The histopathology did

not show any evidence of vasculitis, tubulointerstitial

compartment showed marked sclerosis of the interstitium

and scattered moderate inflammatory cells, tubular atro-

phy with dilatation. The features were consistent with the

end stage renal disease (Figure 3)

Even the lung tissue biopsy done did not reveal any

granuloma, pneumonic changes and evidence of vascu-

litis. However there were findings of scanty recent and

Figure 1. Chest X-ray showing nodular opacities (indicated

by small and big arrows).

Figure 2. CT scan of the chest showing ground glass ap-

pearance.

old intra-alveolar haemorrhage (5% of the tissue sample)

(Figure 4)

Semi-permanent vascular access (Right Internal Jugu-

lar vein tunneled catheter) was inserted for him and he

was put on maintenance haemodialysis three times per

week and radiocephalic fistula was also created for him.

He was discharged to the peripheral health care facility

with dialysis facility, in a stable condition with a plan for

living related renal transplantation from his brother in the

future.

3. Discussion

The presentation with pulmonary oedema is very com-

mon in patients on maintenance haemodialysis. These

end stage renal disease patients irrespective of etiology

are at times polyuric before being started on dialysis.

However after initiating on haemodialysis they tend to

decrease their urine output. Little excess of fluid intake

inter dialytic period leads to pulmonary congestion an d at

times frank pulmonary oedema. This is exacerbated by

the concur re nt presenc e of poorly functioning heart.

In our patient the scenario was a bit perplexing as he

presented for the first time with very advanced renal

failure and pulmonary congestion. However his urine

Figure 3. Haematoxylin Eosin stain section shows a core of

kidney tissue with two globally hyalinised glomeruli, (black

arrow heads) marked tubulointerstitial sclerosis and mild

chronic infl a mmation.

Figure 4. Section showing the lung tissue with mild thick-

ening of the alveolar septa and congestion. No vasculitis or

granuloma seen.

Open Access OJNeph

M. BUDRUDDIN ET AL.

Open Access OJNeph

183

output was nearly 1500 ml in 24 Hours. The clinical pre-

sence of frank haemoptysis with first time documentation

of grossly deranged renal functions and CT finding of

alveolar haemorrhage was quite suggestive of pulmonary

renal syndrome (Wegener’s Granulomatosis/Good pas-

tures syn drome).

Pulmonary oedema can at times present with haemop-

tysis but again it usually does not show alveolar haemor-

rhages.

However going through the literature we realized that

radiological findings of pulmonary oedema can present

in several forms [1].

a) Postobstructive pulmonary edema.

b) Pulmonary edema with chronic pulmonary embo-

lism.

c) Pulmonary edema with veno-occlusive.

d) Stage 1 near drowning pulmonary edema manifests

as Kerley lines, peribronchial cuffing, and patchy,

perihilar alveolar areas of airspace consolidation;

e) stage 2 and 3 lesions are radiologically nonspecific.

f) Pulmonary edema following administration of cyto-

kines.

g) High-altitude pulmonary edema.

(h) Neurogenic pulmonary edema manifests as bilateral,

rather homogeneous airspace consolidations that pre-

dominate at the apices in about 50% of cases.

i) Reperfusion pulmonary edema.

j) Postreduction pulmonary edema manifests as mild

airspace consolidation involving the ipsilateral lung.

k) Pulmonary edema due to air embolism initially de-

monstrates interstitial edema followed by bilateral, peri-

pheral alveolar areas of increased opacity that predomi-

nate at the lung bases.

The typical alveolar shadows seen in our patients CT

is one of the forms.

Further it is well known that those patients with ob-

structive airway disease who develop congestive heart

failure have much higher pulmonary extravascular vol-

ume. Left ventricular failure due to hypoxia leads to left

atrial hypertension, which could be the patho-physiolo-

gic basis of interstitial pulmonary oedema [2].

Histological studies in uremic lungs have proven the

presence of proteinaceous oedema fluid though many

have even shown intra-alveolar haemorrhage [3]. Evalu-

ation of the oedema fluid by direct endotracheal aspira-

tion have even shown high content of protein which

could suggest increased pulmonary vascular p ermeability

to plasma proteins in ren al failure [4]. Though a co mmon

feature of pulmonary oedema is increased permeability

of the basement membrane to water and low molecular

weight solutes, this may be attributable to the hemody-

namic factors playing role in the alveolar interstitium and

basement membrane [5].

4. Conclusion

Patients with pulmonary edema may present not only

blood tinged frothy sputum but may even present mas-

sive haemoptysis, which may be life threatening and

mimicking serious medical conditions, clinically and ra-

diologically. Keeping this in mind one must deal cau-

tiously with patients presenting pulmonary haemorrhage

(haemoptysis) and advanced renal failure.

REFERENCES

[1] T. Gluecker, P. Capasso, P. Schnyder, F. Gudinchet,

M.-D. Schaller, J.-P. Revelly and R. Chiolero, “Clinical

and Radiologic Features of Pulmonary Edema,” Radio

Graphics, Vol. 19, No. 6, 1999, pp. 1507-1531.

http://dx.doi.org/10.1148/radiographics.19.6.g99no21150

[2] R. M. McCredie, “Pulmonary Oedema in Lung Disease,”

British Heart Journal, Vol. 32, No. 1, 1970, pp. 66-70.

http://dx.doi.org/10.1136/hrt.32.1.66

[3] L. Doniach, “Uremic Oedema of the Lungs,” The Ame-

rican Journal of Roentgenology, Radium Therapy and

Nuclear Medicine, Vol. 58, No. 5, 1947, pp. 620-628.

[4] E. C. Rackow, I. A. Fein, C. Sprung, R. S. Grodman,

“Uremic Pulmonary Oedema,” American Journal of Me-

dicine, Vol. 64, No. 6, 1978, pp. 1084-1088.

[5] D. G. Gibson, “Hemodynamic Factors in the Develop-

ment of Acute Pulmonary Oedema in Renal Failure,” The

Lancet, Vol. 288, No. 7475, 1966, pp. 1217-1220.

http://dx.doi.org/10.1016/S0140-6736(66)92302-6