Chief complaints
Blood-tinged sputum
for 2 months and fever, cough for 10 days
This 59-year-old male
patient began to develop blood-tinged sputum in Aug 2000, but
he dud not pay much attention to it. Unfortunately, fever and
productive cough occurred in early October and he visited a
local clinic but the symptoms persisted. He was admitted to
another hospital on Oct 6, 2000. The chest radiograph showed
infiltrates over bilateral lung fields ( Figure 1). Cefmetazole and
gentamicin were given, and were shifted to Rocephin and
Erythromycin due to persistent high fever. Repeated sputum
examinations were negative for acid-fast bacilli. Because his
condition did not improve, he was transferred to the emergency
room of the National Taiwan University Hospital on Oct 12,
2000.
The patient was a heavy
smoker for 40 years and had suffered from chronic cough for 6
months. The past history was otherwise unremarkable.
On examinations, the body
temperature was 37℃, blood pressure 130/76 mm Hg, pulse rate
72 per minute, and the respiratory rate 18 per minute. His
consciousness was clear. The conjunctivae were not pale and
sclera was anicteric. Light reflex of the pupils was prompt
and symmetric. The neck was supple without lymphadenopathy.
The chest wall was symmetric and crackles could be heard over
bilateral lung fields. The heart sounds were regular, without
murmurs. The abdomen was flat and soft. There was no
tenderness or rebound tenderness. The liver and spleen were
not palpable. The extremities were movable and without
cyanosis or edema. All peripheral pulses were intact.
The follow-up CxR and Chest
CT scans showed multiple pneumonic patches of bilateral lung
fields (Figure 2、3、4).
<Lab >
1. Urinary analysis
|
Protein |
Bilirubin |
Urobil. |
RBC |
WBC |
Epith. |
Cast |
89/10/16 |
30 |
-- |
0.1 |
10-15 |
0-2 |
0-1 |
RBC |
2. CBC
|
WBC |
RBC |
PLT |
HB |
MCV |
Seg. |
Lymph |
Mono. |
89/10/13 |
20800 |
3.15 |
623 |
9.9 |
89.5 |
87.0 |
6.0 |
|
89/10/18 |
20900 |
3.22 |
455 |
10.0 |
91.0 |
90.2 |
3.3 |
|
89/10/20 |
22860 |
2.89 |
178 |
8.9 |
|
|
|
|
89/10/23 |
27780 |
2.81 |
39 |
8.2 |
|
|
|
|
89/10/26 |
40040 |
4.3 |
45 |
12.9 |
|
|
|
|
3. BCS
|
Alb |
Glo |
T-Bil |
D-Bil |
ALP |
GOT |
GPT |
BUN |
Cre |
Na |
K |
891013 |
2.2 |
3.6 |
0.9 |
|
167 |
60 |
30 |
36 |
2.2 |
125 |
4.9 |
891019 |
1.0 |
1.6 |
0.9 |
0.6 |
85 |
116 |
|
87 |
2.6 |
139 |
4.9 |
4. Sputum Study:
Date |
Gram's |
AFS |
Cytology |
891020 |
Neg. |
Neg. |
Neg. |
891027 |
Neg. |
Neg, |
Neg. |
5. Clotting & DIC profiles:
|
PT |
PTT |
3P |
FDP |
D-Dimer |
Fibrinogen |
Date |
sec |
sec |
|
μg/ml |
μg/ml |
mg/dl |
891018 |
18/12 |
40/38 |
Neg. |
40-80 |
1.33 |
170 |
6. Blood pH/Gas
項 目: |
pH |
PCO2 |
PO2 |
HCO3 |
BE |
日期 |
|
mmHg |
mmHg |
mEq/l |
mEq/l |
891013 |
7.42 |
37.8 |
119.4 |
23.9 |
0.0 |
891016 |
7.17 |
71.8 |
49.3 |
24.9 |
-4.1 |
891019 |
7.42 |
43.3 |
58.9 |
27.8 |
3.5 |
7. Pleural effusion study
Date |
WBC |
L:N:M |
Gram |
AFS |
TP |
Sugar |
LDH |
10/13 |
1900 |
47:52:1 |
Neg |
Neg |
3.5 |
119 |
|
10/23 |
500 |
31:51:18 |
Neg |
Neg |
2.3 |
133 |
1770 |
8. Abdominal echo showed only parenchymal renal disease.
9. Viral marker: HBsAg(-), Anti-HBs(+), Anti-HCV(-)
10. Serology:
ANA 1:40 |
Anti-basement memb. Ab(-) |
CRP: 20.8 mg/dl |
RA factor 1:10240 |
C3: 78.7 mg/dl |
C4: 9.8 mg/dl |
IgA 306. mg/dl |
IgG 1340 mg/dl |
IgM 64.3 mg/dl |
Cryoglobulin: Negative |
Antiphospholipid Ab: Negative |
Legionella Urinary Ag: Negative |
ANCA: |
PR3 (c-ANCA): 24.8 (positive) |
|
MPO (p-ANCA): 1.5 (negative) |
|
CMV Ab: 1:2 (--) |
11. Blood culture: No growth
12. Bronchoscopy (89-10-19): Diffuse inflammatory mucosa.
Much fresh blood and frothy sputum are noticed over both
bronchial trees. There is no endobronchial lesion.
Bronchoalveolar Lavage: |
PMN: 95.2%, macrophage: 2.6%, |
|
eosinophil: 0.4%, lymphocyte: 0.6% |
|
Hemosiderin-laden macrophage (+) |
|
Culture: No growth |
13. Pathology:Lung biopsy: diffuse acute capillaritis,
marked pleuritis with interstitial granuloma nodules and
neutrophils infiltration.
<COURSE and Treatment
>
After admission, massive
hemoptysis with acute respiratory failure developed on Oct 19
and the CxR lesions also deteriorated (Figure 5). He was intubated and was
transferred to the MICU. Bronchoscopy showing large amount of
frank bloody and frothy airway secretion from both lungs.
Steroid pulse therapy (methyloprednisolon, 1000 mg/day x 3
days) and plasmaphoresis were started on Oct 21.
Unfortunately, poor oxygenation persisted even under high PEEP
and 100% of oxygen. Extracorporeal membrane oxygenation (ECMO)
supportive system was used on Oct 22. Cyclophasphamide (750
mg/day x 1 day) was also given on Oct 25 because of
deterioration of renal function and oligouria. Unfortunately,
fever flared up on Oct 30 and the blood and sputum cultures
yielded Acintobacter baumannii. Repeat steroid pulse therapy
was continued but there was no clinical improvement. The
patient passed away on Nov 2, 2000.
案例分析
本病例產生高燒,兩側之肺部浸潤、咳血,且尿中有紅血球柱體。應聯想到有可能產生肺腎症候群(pulmonary-renal
syndrome)。此類疾病中包含了一些全身性之血管炎。由臨床表徵要得到確切診斷有時不太容易,需要血清學及病理之診斷輔助證據。Wegner's
granulomatosis有時臨床表現並不具有典型之器官侵犯(上呼吸迫,肺及腎臟),有時只有局部表現 (limited
form),但隨著病情進展幾乎都會侵犯肺及腎臟。本病例屬嚴重型之Wegner's
granulomatosis。雖然經大量類固醇及免疫抑制劑治療仍無法改善、最終因敗血症及多器官衰竭而死亡。
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