網路內科繼續教育
有效期間:民國 94年07月01日 94年07月15日

    Case Discussion

主訴(Principal Description):

Dry cough with intermittent fever and progressive dyspnea for 1 month

病史(Brief History):

The 27-year-old man was a previously healthy student. He suffered from traumatic fracture of right fibula and tibia in a traffic accident 2 months ago and received only traditional bone manipulation. About 1 month ago, he began to develop a dry cough. Fever occurred several days later but there was only mild dyspnea. He visited the ER of a medical center in Taipei on April 8th, 2005. He did not have myalgia, anorexia or diarrhea. The CxR revealed bilateral interstitial infiltrates. The WBC count was 21600, with 84% of neutrophils. He was then admitted and antibiotics (Augmentin and Zithromax) were given. His fever gradually subsided and cough almost resolved, but the CxR lesions did not improve. The sputum acid-fast staining was negative. He was discharged on April 13 and continued to take oral antibiotic (levofloxacin). Nine days after discharge, however, fever relapsed and he was sent back to the ER. Some IV antibiotics were administered and the fever subsided in 2 days. Unfortunately, his cough and fever recurred on May 1. The CxR showed diffuse reticular pattern, especially in the right upper lung field. Morxifloxacin was given, and fever subsided in 2 days and his cough also improved. However, progressive dyspnea developed after discharge. It progressed rapidly in 3-4 days, and nonproductive cough also worsened. He went to the ER in another medical center on May 6 where mild hypoxemia was found. The CxR (Figure 1) and Chest CT (Figure 2 ) showed interstitial infiltrates and patchy consolidations over the right lung.

<Past History>

  1. Denied systemic disease;
  2. Smoking(-),Alcohol(-)
  3. Denied drug allergy, allergy to sea food;
  4. Surgical history: nil
  5. Occupation:student

體檢發現(Evident Physical findings):

    Conscious: clear and alert
    Vital sign: 36.8 0 C/100/19 BP: 137/55 mmHg
    HEENT: Conjunctiva: not pale, Sclera: anicteric
    Pupil: isocoric, 3mm/3mm, L/R +/+, prompt
    Neck: supple, no LAP, JVE (-), no bruit
    Chest: symmetric expansion
    Heart: RHB, no murmur
    Abdomen: soft and flat, no tenderness, no palpable mass
    Liver: palpable, 4cm below RCM
    Spleen: impalpable
    Bowel sound: normoactive; Operation scar: nil
    Extremity: no edema, normal skin turgor, no petechia or ecchymosis
    Healed abrasion ulcers at right leg and ankle
    Skin: normal skin turgor
    LAP: no axillary LAP, no inguinal LAP, no supraclavicular LAP

手術方法及日期(Operation):

94-05-13 VATS biopsy (Lung,middle and upper lobes, right): Patho: bronchiolitis obliterans-organizing pneumonia (BOOP); with plugs of spindle mesendymal cells obstruct the air spaces and bronchioles.

住院治療經過(Course and Management):

After admission, he developed several episodes of severe dyspnea and IV steroid was given, with good response. Bronchoscopy and BAL was performed. Video-assisted thoracoscopic surgery (VATS) biopsy on May 13 confirmed the diagnosis of BOOP (Figure 3). IV corticosteroid was given and his dyspnea and arterial oxygenation improved quickly. He was then discharged on May 18, with continued oral prednisolone therapy (1 mg/day). The follow-up CxR 2 weeks after discharge (Figure 4 ) was almost clear.

Laboratory findings:

  WBC /mm3 Hb g/dL MCV /fL Hct % Plt K/μL
05/06 20750 13.2 88 40.2 483
05/08 14910 12.2 86.9 37 511

   Alb g/dL  T-bil g/dL GOT U/L GPT U/L ALP U/L  γ-GT U/L LDH U/L
05/06 4.0 0.57 83 130 625 259 470

CK U/L BUN mg/dL Cr mg/dL  Na mM K mM CRP mg/l
53 5.8 0.8 134 4.3 23.2

Anti-DNA(FA)  1:10 -
Anti-ENA(Jo-1)  Negative
Anti-ENA(RNP) Negative
Anti-ENA(SCL-70) Negative
Anti-ENA(SM) Negative
Anti-ENA(SSA) Negative
Anti-ENA(SSB) Negative
Anti-ENA(Scl-70) Negative 
Anti-ENA(Sm) Negative
Anti-Nuclear Antibody 1:80 + Speckle
C3 Quantitation 139.0
C4 Quantitation 9.8
IgA 258.0 (mg/dl)
 IgG 1560.0 (mg/dl)
IgM 102.0 (mg/dl)
 RA Factor (Nephelometry) < 20.0 (IU/mL)
Mycoplasma Pneumoniae IgM Ab Negative

細菌室(BA)

BRONCHIAL WASHING:       Common Pathogens
                                                  1: (2+) Neisseria species
                                                  2: (2+) Viridans streptococci
PLEURAL EFFUSION             No aerobic&anaerobic pathogens
Lung biopsy : No aerobic&anaerobic pathogens:, No fungus
BRONCHIAL WASHING       Acid fast bacilli - negative
BRONCHOALVEOLAR LAVAGE        Acid fast bacilli - negative

病理報告(Pathology Report):

Bronchiolitis obliterans-organizing pneumonia (BOOP)

Pulmonary function:
Flow-Volume Test (BTPS, Sitting Position)
  Observed Predicted % Predicted Post-Test
FVC (L) 1.97 4.05 48.7 1.69
FEV1.0(L) 1.41 3.59 39.3 1.29
% FEV1.0 (%) 71.6 87.0   76.33

  Observed Predicted % Predicted Post-Test
VC 1.97 4.05 48.7 1.71
IC 1.28     1.05
ERV 0.69 1.69 40.8 0.66
FRC 2.11 3.15 67.1 1.95
RV 1.23 1.24 98.9 1.45
TLC 3.19 5.29 60.3 3.16
RV/TLC(%) 38.6 23.4   45.89

Diffusion Capacity (ml/min/mmHg, STPD)
  Observed  Predicted % Predicted
DLCO 11.02 29.21 37.72

本病例為一位原來身體健康良好之青年。主要的表現為乾咳及發燒等症狀,經過胸腔X光片發現肺葉有疑似肺炎的病灶,於是在一開始被診斷為一般細菌引起的社區性肺炎治療,但是效果並不理想。後來經過切片才診斷為阻塞性細支氣管炎合併器質化肺炎(bronchiolitis obliterans with organizing pneumonia, BOOP)。在使用類固醇藥物後,病人的症狀很快得到了改善。

器質化肺炎是肺臟針對許多不同病因所造成急性損害的一種修護反應,但是病變只侷限於小氣道、肺泡管、及細支氣管旁肺泡等。BOOP是一種特殊的疾病分類,具有一些獨特的臨床及病理特徵。病因大多不明,可與藥物、器官移植有關。臨床上是一種類似肺炎的疾病,但病理上顯示小氣道的管腔被纖維化或肉芽組織填塞,而這些纖維化或肉芽組織會擴展至肺泡管(alveolar ducts)及肺泡(alveoli)。由於細支氣管的管腔被阻塞,所以,也稱之為阻塞性細支氣管炎合併器質化肺炎(bronchiolitis obliterans with organizing pneumonia, BOOP)。本病例為一典型病例。在臨床上懷疑肺炎的病人,當有一個慢性的病程,同時又對一般抗生素無效時,必須要考慮其他原因引起之肺實質化。

繼續教育考題
1.
(D)
Typical idiopathic BOOP is characterized by the following EXCEPT:
AFlu-like illness
BBilateral fine crackles,
CPatchy infiltrates on CxR
DLoud wheezing
2.
(D)
The pathological features of BOOP are similar to those observed in:
AObliterative bronchiolitis (BO)
BUsual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF).
CSarcoidosis
DNone of the above
3.
(D)
Drug-related BOOP has been reported from use of several different types of medications, including:
ABleomycin sulfate
BAmiodarone
CMethotrexate
DAll are correct
4.
(C)
Which statement about BOOP is WRONG ?
ABOOP has been reported with all of the connective tissue diseases
B BOOP might be the first manifestation of a connective disorder
CBOOP has not been reported in patients with bone marrow transplantation
DBOOP has been reported with common variable immunodeficiency syndrome and essential mixed cryoglobulinemia
5.
(A)
Which description about the pulmonary function in BOOP is WRONG ?
AUsually associated with severe airway obstruction
BFlow rates are usually normal except in smokers.
CDiffusing capacity (DLco) is decreased in almost all patients
DThe vital capacity is usually reduced
6.
(B)
Which is the most common type of BOOP ?
ADrug-related BOOP
BIdiopathic BOOP
CBOOP associated with connective tissue disorders
DBOOP after lung transplantation
7.
(C)
Which of the following is the drug of choice for BOOP ?
AIsoniazid
BIVIG
CPrednisolone
DImuran
8.
(D)
The recommended duration of drug therapy for BOOP is :
A2 weeks
B1 month
C4 month
D6-12 months
9.
(B)
Which of the following description about BOOP is WRONG ?
ABOOP is a treatable inflammatory lung disease
BThe prognosis of idiopathic BOOP remains poor
CIn patients treated for < 1 year, BOOP might recur in one third.
DRelapsed BOOP can usually be successfully treated with the previously responsive dosage level
10.
(A)
Which statement about pathological features is RIGHT ?
APolyploid endobronchial connective tissue masses filling the lumens of terminal and respiratory bronchioles and alveolar ducts
BHemophagocytosis
CHoneycombing
DGray hepatization

答案解說
  1. (D) Typical idiopathic BOOP is characterized by a flulike illness, bilateral crackles, and patchy infiltrates
  2. (D ) Please see the explanation of question 10.
  3. (D) Drug-related BOOP has been reported from use of several different types of medications, including anti-inflammatory and immunosuppressive agents such as bleomycin sulfate, gold, and methotrexate; antibiotics such as sulfasalazine, sulfamethoxypyridazine, cephalosporins, amphotericin B and amiodarone.
  4. (C ) BOOP can be associated with dermatomyositis ankylosing spondylitis, polymyalgia rheumatica, and Behcet disease and might be the first manifestation of a connective disorder. BOOP has been reported with common variable immunodeficiency syndrome and essential mixed cryoglobulinemia. BOOP has been described in patients who underwent marrow transplantation. It remains unclear whether BOOP in these patients is an incidental finding or represents a complication of bone marrow transplantation.
  5. (A ) Results of pulmonary function studies show decreased vital capacity. The flow rates are normal except in smokers. The diffusing capacity is decreased in almost all patients.
  6. (B ) Idiopathic BOOP is the most common type of BOOP.
  7. (C) Prednisone continues to be recommended as first-line treatment for patients with symptomatic and progressive disease. The dosage is generally 1 mg/kg (60 mg/d) for 1 to 3 months, then 40 mg/d for 3 months, then 10 to 20 mg/d or every other day for a total of 1 year. Every-other-day scheduling can be successfully used for this disorder. A shorter 6-month course may be sufficient in certain situations.
  8. (D ) The recommended duration of drug therapy for BOOP is :6012 months
  9. (B) The prognosis of idiopathic BOOP remains good. In patients treated for less than 1 year, BOOP might recur in one third. It is a lung disorder that can be successfully treated a second and third time with the previously responsive dosage level of prednisone. Relapse of BOOP may be related to the severity of the illness.
  10. (A ) BOOP is characterized by polyploid endobronchial connective tissue masses filling the lumens of terminal and respiratory bronchioles and extending into alveolar ducts and alveoli, representing an organizing pneumonia.


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