A pulmonary and critical care pearls Dyspnea and Hemoptysis Develop in a

pulmonary and critical care pearls
Dyspnea and Hemoptysis Develop in a
Young Man With Prostatitis*
Carolina Q. See, MD; H. Ari Jaffe, MD; and Dean E. Schraufnagel, MD, FCCP
(CHEST 2005; 128:3625–3628)
31-year-old man was admitted to the hospital
A with
hemoptysis and progressive dyspnea. He
was well until 4 months prior to hospital admission,
when night sweats, anorexia, fevers, and 30-lb weight
loss developed. Two months prior to hospital admission, he began to experience rectal pain, dysuria, and
hematuria. One month prior to hospital admission,
he presented with acute urinary retention at the
emergency department. His prostate on digital rectal
examination was enlarged and tender, and a CT of
the pelvis suggested that he had prostatic abscess. A
transurethral prostatic resection uncovered pockets
of pus and friable tissues. Microscopically, acute and
chronic necrotizing prostatitis was seen (Fig 1) He
was discharged home on oral antibiotics.
Over the next 2 weeks, the patient had blurred
vision, hearing loss, and myalgias. He returned to the
emergency department and was administered ocular
tobramycin. Findings of a chest radiograph done at
that time were normal.
Four days prior to hospital admission, bloodtinged sputum and shortness of breath developed.
This progressed, and the patient was admitted to the
hospital.
The history of the patient revealed that he immigrated to the United States 12 years ago from
Ecuador and had not traveled since then. He denied
smoking, alcohol, or IV drug use. He also denied a
history of tuberculosis or known exposure to tuberculosis. Results of a tuberculin skin test done 1
month before hospital admission were negative.
*From the Section of Respiratory and Critical Care Medicine,
Department of Medicine, University of Illinois at Chicago Hospital, Chicago, IL.
Manuscript received January 19, 2005; revision accepted March
7, 2005.
Reproduction of this article is prohibited without written permission
from the American College of Chest Physicians (www.chestjournal.
org/misc/reprints.shtml).
Correspondence to: Carolina Q. See, MD, Section of Respiratory
and Critical Care Medicine, Department of Medicine, University
of Illinois at Chicago Hospital, 840 South Wood St, M/C 719,
Chicago, IL 60612-7323
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Figure 1. The histopathology of prostate shows acute and
chronic inflammation (hematoxylin-eosin, low-power field).
At the time of hospital admission, the patient was
in mild respiratory distress and tachycardic. Examination of the chest was normal. Lateral episcleritis
was noted in both eyes. His nasal turbinates were
clear, and tympanic membranes were normal. Neurologic examination was grossly symmetrical and
intact. Admitting laboratory data revealed hemoglobin of 8.2 g/dL, mean corpuscular volume of 78
femtoliters, a leukocyte count of 11,000/mL with
84% neutrophils, 8% lymphocytes, 5% monocytes,
2% eosinophils, and 1% basophils. The platelet count
was 459,000/mL. With no supplemental oxygen, the
arterial pH was 7.51, Paco2 was 29 mm Hg, and
Pao2 was 53 mm Hg. The BUN was 8 mg/dL, and
creatinine was 0.7 mg/dL. The urinalysis showed
“large” blood, “trace protein,” 40 RBCs per lowpower field, and 18 WBCs per low-power field. A
chest radiograph showed the development of bilateral upper lobe alveolar opacities that were not
present 1 week before (Fig 2).
The patient was placed on respiratory isolation,
and hypoxemic respiratory failure rapidly developed,
CHEST / 128 / 5 / NOVEMBER, 2005
3625
Figure 2. The chest radiograph taken at the time of hospital
admission shows bilateral alveolar opacification with air bronchograms.
requiring mechanical ventilation. His repeat chest
radiograph showed rapid and extensive progression
to nearly complete alveolar opacification (“whiteout”) with air bronchograms.
What is the diagnosis?
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Pulmonary and Critical Care Pearls
Diagnosis: Wegener granulomatosis with diffuse
alveolar hemorrhage and prostatitis
First described in 1936, Wegener granulomatosis
is a clinical syndrome that consists of necrotizing
granulomatous vasculitis of the upper respiratory
tract, lungs, and kidneys. The limited form of Wegener granulomatosis spares the kidney. Wegener
originally described it as a rhinogenic form of polyarteritis nodosa due to its prominent nasal and
paranasal involvement. Although the clinical attention has been focused mainly on the respiratory and
renal systems, Wegener granulomatosis has been
known to involve almost any organ in the body,
including the eyes, ears, heart, skin, joints, peripheral and central nervous systems, and lower genitourinary tract. It can affect persons of all ages but is
more common in middle-aged patients, predominantly men.
An immunologic pathogenesis has been implicated
because of the findings of hypergammaglobulinemia
and circulating antibodies, particularly anti-neutrophil cytoplasmic antibodies in patients with this
disorder. The hypothesis is that the antibodies induce a necrotizing vasculitis by inciting a respiratory
burst and degranulation of neutrophils and monocytes. These initial events require the priming of
leukocytes by cytokines, leading to the expression of
proteinase 3 and myeloperoxidase on the cell surface.
Lung involvement occurs in 80% of cases of
Wegener granulomatosis. Its involvement may range
from minimal to life threatening. Symptoms are
nonspecific and include cough, dyspnea, hemoptysis,
and pleuritic chest pain. Cough is usually nonproductive and is the most frequent symptom. Dyspnea
occurs particularly with diffuse alveolar hemorrhage
and also in the rare setting of tracheal involvement.
Pulmonary capillaritis is seen in approximately one
third of patients with lung disease and may lead to
diffuse alveolar hemorrhage, hemoptysis, and rapidly
changing alveolar opacities on chest radiograph.
Although the lung is the most common organ
system affected in Wegener granulomatosis, diffuse
alveolar hemorrhage as a result of capillaritis is
uncommonly seen. Hemoptysis, if it occurs, is usually related to nodules, inflammatory, or necrotic
lesions, rather than diffuse alveolar hemorrhage. In
the largest surgical pathologic series of Wegener
granulomatosis, alveolar hemorrhage was the dominant pathologic finding in only 7% of all 87 specimens. When diffuse alveolar hemorrhage is present,
it is often the initial manifestation and is usually
associated with renal involvement. It may rapidly
progress to respiratory failure with a mortality of
approximately 50%. On the chest radiograph, a
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diffuse, bilateral alveolar filling pattern is seen. Our
patient did not initially present with diffuse alveolar
hemorrhage and did not have renal impairment.
Diffuse alveolar hemorrhage is a cause of elevated
carbon monoxide diffusing capacity. However, this
test is usually not performed, as dyspneic patients in
the intensive care setting poorly tolerate it. The
findings of flexible fiberoptic bronchoscopy with
BAL are distinctive in alveolar hemorrhage. The
lavage fluid is bloody, and iron stains of bronchoalveolar specimens show hemosiderin-laden macrophages indicative of chronic hemorrhage.
Kidney involvement is common and usually occurs
within 2 years of the initial upper airway disease.
Involvement of the lower urogenital tract in Wegener granulomatosis is rare. It may manifest itself as
acute urinary retention or prostatitis, orchitis, ureteral stenosis, bladder pseudotumor, and penile ulceration. Prostatitis is the most common manifestation of lower urogenital disease, causing urinary
frequency, dysuria, gross hematuria, and urinary
retention. However, it is usually asymptomatic;
symptomatic prostatitis is reported to occur between
2.3% and 7.4% of patients with urogenital involvement. Urogenital symptoms usually resolve promptly
with high-dose corticosteroids and immunosuppressive drugs such as cyclophosphamide.
In a review of 80 patients with Wegener granulomatosis, the main pulmonary features of nodular
opacities usually were combined with urogenital
symptoms. We could find no report on the combination of prostatitis and diffuse alveolar hemorrhage
in the literature.
The diagnosis of Wegener granulomatosis is usually made by the histologic demonstration of three
essential findings: small- and medium-vessel vasculitis, patchy or geographic necrosis, and granulomatous inflammation with neutrophils, lymphocytes,
plasma cells, macrophages, giant cells, and eosinophils. The prostatic biopsy specimen obtained from
our patient showed all of these features. The organ
site and the amount of tissue obtained influence the
likelihood of obtaining a positive biopsy result. The
yield from upper airway biopsies is typically lower
than those obtained from the lungs.
Life-threatening Wegener granulomatosis requires urgent treatment with high doses of glucocorticoids and cyclophosphamide. The medications are
usually continued for at least 1 year after remission.
For patients who have an exacerbation of the disease,
long-term use of the least toxic drug for the maintenance of remission may be appropriate. This may
include methotrexate, azathioprine, and low-dose
prednisone.
Our patient presented initially with extrapulmonary manifestations, including prostatitis, transient
CHEST / 128 / 5 / NOVEMBER, 2005
3627
vision, and hearing loss followed by fulminant hypoxemic respiratory failure secondary to diffuse alveolar
hemorrhage. The initial differential diagnosis included bacterial, mycobacterial, and fungal pneumonia in addition to vasculitis. His treatment included
antimicrobials to bacteria and tuberculosis and corticosteroids.
Bronchoscopic examination showed normal airways. The BAL revealed frank blood and a predominance of neutrophils. Results of bacterial, fungal,
mycobacterial, and viral cultures and special stains
were negative. Serum antinuclear and anti-glomerular basement membrane antibodies, and rheumatoid
factor were negative. His tuberculin skin test was
negative, and anti-neutrophil cytoplasm antibodies
were positive. The clinical manifestation of hemoptysis, dyspnea, iron deficiency anemia, and a bilateral
air space filling pattern on chest radiography were
consistent with the syndrome of diffuse alveolar
hemorrhage. Cyclophosphamide was added, and antimicrobials were discontinued. The patient improved rapidly over 14 days, was extubated, and was
ultimately discharged breathing well on room air. He
remained on cyclophosphamide and a reduced dose
Figure 3. The chest radiograph at the time of discharge showed
impressive improvement of the lesions. Residual alveolar opacification still obscures much of the left hemidiaphragm. Alveolar
and ground glass opacification are also present, especially in the
right mid-lung. The costophrenic angles are blunted.
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of corticosteroids. Figure 3 shows his chest radiograph on discharge from the hospital.
Clinical Pearls
1. The diagnosis of Wegener granulomatosis
should be considered in patients who present with
seemingly disparate multiorgan manifestations, especially when the respiratory, urogenital, ophthalmic,
and otic systems are involved.
2. Symptomatic involvement of the lower genitourinary tract in Wegener granulomatosis, although
uncommon, may sometimes precede the onset of
pulmonary disease.
3. Diffuse alveolar hemorrhage in Wegener granulomatosis is a life-threatening event that responds to
steroids and cyclophosphamide.
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Pulmonary and Critical Care Pearls