10. CXR28:
Aneurysm Arch of Aorta
Leaking Blood into Pleural Space
• Mediastinal mass
• Calcification of periphery evident along upper margin
• Loss of silhouettes of
– aortic knob
– left heart margin
– left diaphragm
• Left pleural effusion
• Tracheal indentation
• Lateral view
• Middle mediastinal mass
• Calcification of periphery in lateral view
• left diaphragm not visible due to fluid in pleural space
Saturday, December 22, 2012
12. Pectus Excavatum
• Indistinct right heart margin.
• Vague density in right lower lung field
• Lateral view
• reveals protruding sternum and flattening
heart
Saturday, December 22, 2012
16. Pulmonary Sequestration
• Anomalous Arterialization of Lung:
• Increased vascular markings at right base
• Smaller right pulmonary artery
Big anomalous artery arising from aorta supplying right lung
Saturday, December 22, 2012
18. chest clinical cases
Dry Cough and Clubbing
in a 45-Year-Old Woman
Submitted by
Jamie L. Garfield, MD
Fellow
Pulmonary and Critical Care Medicine
Temple University School of Medicine
Philadelphia, Pennsylvania
Victor Kim, MD
Assistant Professor of Medicine
Temple University School of Medicine
Philadelphia, Pennsylvania
19. History
• A 45-year-old white female presents to pulmonary clinic for evaluation of increasing chest
tightness, cough and dyspnea.
• She has a history of chronic respiratory symptoms since childhood and frequent exacerbations of
bronchitis, but no history of respiratory failure.
• She reports that until 1 year ago she was limited by mild pleuritic chest tightness and shortness of
breath with heavy exertion.
• She now reports daily symptoms of fatigue, dyspnea, and exercise intolerance with minimal
exertion.
• She has persistent dry cough and occasionally expectorates granular mucus.
• She has been using 3 liters per minute of supplemental oxygen via nasal cannula for the past year.
• Her past medical history is significant for polycythemia, gastroesophageal reflux disease,
fibronodular breast disease, chronic tinnitus with hearing loss and genital herpes.
• Her current medications include famotidine, diltiazem, valacyclovir, inhaled fluticasone,
salmeterol and albuterol.
• She has a 30 pack-year smoking history, having quit smoking 5 years ago.
• She drinks 1-2 beers per week and she denies illicit drug use.
• She is a homemaker and reports no occupational exposures.
• Her parents, siblings and 2 children (ages 16 and 24) are alive and well with no respiratory disease
or malignancy.
Saturday, December 22, 2012
20. Physical Exam
• her heart rate is elevated at 112 beats per minute.
• Oxygen saturation on 2 liters of oxygen via nasal
cannula is 89%.
• Her vital signs are otherwise normal.
• Her head and neck exam is benign.
• Her pulmonary exam is significant for diffuse
inspiratory and expiratory dry crackles, more
prominent at the bases bilaterally.
• Cardiac exam reveals regular rhythm with no murmurs,
rubs or gallops.
• There is clubbing but no cyanosis or edema of her
extremities.
Saturday, December 22, 2012
21. Lab
• White blood cell count 12,900/mm3,
• Hemoglobin 15.5 mg/dl,
• Platelet count 473,000/mm3.
• BUN 9 mg/dl, creatinine 1.0 mg/dl,
• calcium 8.8 mg/dl and phosphate 4.9 mg/dl.
• PT, PTT, electrolytes, glucose and liver function
tests were within normal limits.
• Arterial blood gas on 3L/min supplemental
oxygen revealed a
– pH of 7.39, PCO2 40 mm Hg and PaO2 62 mm Hg.
Saturday, December 22, 2012
22. Studies
• Echocardiogram:
– left ventricular ejection fraction was 55-65% and
– grade 1 diastolic dysfunction was noted;
– right ventricle was mildly dilated and had mildly depressed systolic function;
– pulmonary artery systolic pressure was estimated to be 48-52 mm Hg; right atrium was
mildly dilated and there was mild tricuspid regurgitation.
• Cardiac Catheterization: PAP: 30/6/13; PCWP: 2; CO: 3.38; CI: 2.16.
• Pulmonary Function Tests:
– FEV1/ FVC: 74%;
– FEV1: 0.96 L, 39%;
– FVC: 1.29L, 42%;
– TLC: 2.27L, 53%; RV: 1.14L, 79%; DLCO: 3.11L, 57%.
• 6-Minute Walk Test:
– Resting SpO2: 86% RA; 92% on 2L/min; 8L/min was required to maintain oxygen saturation
above 90%
– at the end of 6 minutes; the patient was able to walk 211 meters.
Saturday, December 22, 2012
25. Bronchoalveolar lavage
Slightly mucoid, tan fluid; cultures revealed no growth; cytology showed no evidence
of malignancy; a few white blood cells were noted, predominantly macrophages;
laminated calcified concretions and debris were also present.
Von Kossa stain positive.
Saturday, December 22, 2012
26. Question 1
• What is the most likely diagnosis?
• A. Idiopathic pulmonary fibrosis
• B. Pulmonary alveolar proteinosis
• C. Diffuse alveolar hemorrhage syndrome
• D. Pulmonary alveolar microlithiasis
• E. Broncheoloalveolar carcinomatosis
Saturday, December 22, 2012
27. Pulmonary Alveolar Microlithiasis
(PAM)
• is a rare disorder of unknown etiology in which microliths occupy the alveolar space (1).
• The mean age of diagnosis is in the fourth decade of life, although the onset of disease may
occur in childhood (2, 3).
• Idiopathic pulmonary fibrosis (IPF) tends to present later in life than PAM.
• Dyspnea and hypoxemia are common to both disease entities, but the honeycombing
classically seen on HRCT in IPF would not be expected in PAM.
• Pulmonary alveolar proteinosis (PAS), another alveolar filling disorder, is also a rare but
deadly entity.
• Patients with PAS may have dry crackles and clubbing as in PAM.
• The patchy ground glass opacities with a “crazy-paving” pattern described in PAS can
usually be distinguished from the microcalcifications seen on HRCT in PAM. Rarely, “crazy-
paving” can be seen in PAM (4).
• Bronchoalveolar carcinomatosis can radiographically mimic many diseases with alveolar
infiltrates.
• Classically this condition is less diffuse than PAM, and as such, hypoxemia and diffusion
impairment are less common.
• Diffuse alveolar hemorrhage (DAH) can present with hypoxia and diffuse alveolar infiltrates
similar to PAM.
• The onset of DAH is usually more abrupt than PAM.
• While frank hemoptysis can be absent in up to one third of DAH cases, hemosiderin-laden
macrophages are commonly found in BAL.
Saturday, December 22, 2012
28. Question 2
• Which of the following statements is true regarding
pulmonary alveolar microlithiasis?
• A. Dyspnea is described in proportion to the severity of
the radiographic abnormalities.
• B. The diagnosis can only be made by lung biopsy
demonstrating microliths within the alveoli.
• C. Pathologically, the lungs are soft and boggy.
• D. Familial disease is inherited in an autosomal
dominant fashion.
• E. PAM is found in all geographic regions, across all
races, and equally between men and women.
Saturday, December 22, 2012
29. PAM
• is present on all continents with no particular geographic or racial distribution, is
reported equally in males and females, and has been described in all age groups,
but most frequently from birth to age 40.
• A family history was noted in 37% of patients in one large review .
• Simultaneous cases of PAM have been described in siblings, supporting an
autosomal recessive inheritance pattern.
• A hallmark feature of PAM is the striking dissociation between clinical and
radiographic findings.
• Often there is extensive radiographic evidence of disease and only mild
symptomatology
• Children that present with PAM are often asymptomatic.
• The diagnosis can often be made in the absence of a biopsy, with radiographic
imaging demonstrating the classic “sand-storm” appearance.
• Bronchoalveolar lavage may demonstrate laminated calcified concretions.
• Diagnosis can be confirmed with open, transbronchial or transthoracic needle
biopsy.
• Histologically, the characteristic finding is that of bluish-violet bodies of varying
shape and sizes, many of which are fissured with radial cracks .
• On gross pathology, lungs are hard and gritty and do not collapse upon removal
from thorax . Saturday, December 22, 2012
30. Question 3
• PAM may be associated with all of the
following conditions/exposures EXCEPT
which of the following?
• A. Milk-alkali syndrome
• B. Calcium containing powdered tobacco
• C. Inflammatory bowel disease
• D. Printing ink
• E. Renal transplant
Saturday, December 22, 2012
31. discussion
• PAM has been described in many patients with no discernable risk
factors for the disease.
• An increased risk of PAM has been reported in individuals with
exposure to printing ink and among those with a history of
smoking powdered tobacco containing calcium salts .
• Systemic diseases associated with calcium and phosphate
deregulation, such as milk-alkali syndrome renal transplant and
nephrolithiasis have been linked to PAM.
• It is not known if these associations are causational or
coincidental.
• Inflammatory bowel disease, while can be associated with
electrolyte abnormalities related to malabsorption, has not been
described in patients with PAM.
Saturday, December 22, 2012
32. Question 4
• Which of the following signs/symptoms
is not commonly associated with PAM?
• A. Pleural plaques
• B. Pneumothorax
• C. Hemoptysis
• D. Lithoptysis
• E. Emphysema
Saturday, December 22, 2012
33. discussion
• On gross pathology, the pleural surfaces are generally
free, though appear granular owing to the sand-like
particles that can be seen and felt beneath.
• Pleural plaques are not commonly seen in PAM .
• Emphysematous blebs are commonly seen in patients
with PAM and may represent early lung fibrosis.
• Pneumothorax resulting from bleb rupture is not rare .
• Some patients report coughing up blood, while others
describe lithoptysis, or expectorating granular-like
mucus or microliths with coughing.
Saturday, December 22, 2012
34. Question 5
• Which of the following statements is FALSE regarding
the underlying pulmonary process in this patient?
• A. Microliths are derived from the alveolar capillary blood
and not a result of pathological changes in the tissue cells
of the alveoli.
• B. Inborn errors in metabolism at the alveolar interface
leads to local accumulation of calcium salts.
• C. Alveolar hemorrhage is commonly associated with this
disease.
• D. PAM usually occurs in patients without evidence of
systemic derangement in calcium metabolism.
• E. Dysfunction of SLC34A2 may reduce the clearance of
phosphate leading to the formation of microliths.
Saturday, December 22, 2012
35. discussion
• The metabolic abnormality in PAM results in precipitation of calciferous salts in an otherwise
normal alveolus.
• The components of the microliths are therefore thought to be derived from the alveolar capillary
blood and not a result of pathological changes in the tissue cells of the alveoli .
• It has been suggested that inborn errors in metabolism at the alveolar interface lead to increased
alkalinity or mucopolysaccharide deposition, promoting the local accumulation of calcium salts.
• Studies of calcium metabolism in affected patients have been consistently normal, suggesting it is
unlikely that PAM is due to a systemic derangement of calcium metabolism .
• Hemoptysis is not uncommon in PAM when inspissated microliths irritate and erode bronchial
epithelium. Diffuse alveolar hemorrhage, however, is not known to be associated with PAM.
• Homozygous inactivating mutations in the SLC34A2 gene, are present in patients with PAM.
• SLC34A2 encodes a sodium phosphate transporter that is highly expressed in alveolar type II
cells.
• Normally, type II alveolar cells recycle and degrade surfactant and subsequently release
phosphate into the alveolar space.
• Dysfunction of this sodium phosphate transporter may reduce the clearance of phosphate from
the alveolar space, thereby leading to the formation of microliths .
Saturday, December 22, 2012
36. Question 6
• Treatment for PAM currently includes all
of the following except?
• A. Bilateral lung transplant
• B. Gene replacement therapy
• C. Whole lung lavage
• D. Systemic steroids
• E. Dibisphosphonates
Saturday, December 22, 2012
37. discussion
• Various treatment regimens have been explored for PAM, most of which
have not altered the natural course of the disease.
• Borrowing from the therapeutic strategy for PAS, whole lung lavage has
been tried without success in patients with PAM.
• Systemic corticosteroids and bisphosphonates have also been used but
fail to preserve lung function or improve symptoms.
• Bilateral lung transplant remains the only option for patients with severe
symptoms related to PAM .
• There remains very little information on long term survival and whether
the disease will return in the donor lungs .
• As more is learned about the SLC34A2 gene and its role in PAM, there is
growing interest in the possibility of gene replacement therapy to treat
PAM .
• While this therapy is not available today, early data suggest that
targeting phosphate rather than calcium metabolism may be beneficial
for the treatment of PAM.
Saturday, December 22, 2012
43. HRCT-1
• Find an example in the right lung of combined
bronchovascular and interlobular septal
thickening with distortion of the architecture.
• Find an example in the left lung of thickened
bronchovascular interstitium.
• Find a centrilobular nodule, representing
thickening of the terminal bronchiolovascular
interstitium, in the left lung.
46. • Find lymph nodes.
• Find the main pulmonary artery.
• The adjacent thinner-walled vessel is the vein.
• Find and outline a longitudinally-cut, segmental
bronchus with thickened bronchovascular
interstitium that narrows the lumen.
• This bronchovascular interstitial involvement
corresponds to that seen in the HRCT image
above.
• Architectural distortion may result.
• Find other airways cut in cross-section that are
also narrowed.
49. • Find an airway and name its type.
• Find the accompanying pulmonary artery
branch.
• Find and identify 5 rounded, interstitial,
cellular structures.
• Find and identify the composition of the pink
matrix.
50. Histologic Appearance
Bronchiole (airway has no cartilage)
This picture shows thickening of the interstitium.
51. Differential diagnosis
• Differential diagnosis of nodular
bronchovascular and interlobular septal
thickening on HRC:............
• Histologic differential diagnosis:………
52. Differential diagnosis
• Differential diagnosis of nodular
bronchovascular and interlobular septal
thickening on HRC:
–Sarcoidosis.
–lymphangitic tumor.
–Lymphoma.
–Kaposi's sarcoma
• Histologic differential diagnosis:………
53. Differential diagnosis
• Differential diagnosis of nodular bronchovascular and
interlobular septal thickening on HRC:
– Sarcoidosis.
– lymphangitic tumor.
– Lymphoma.
– Kaposi's sarcoma
• Histologic differential diagnosis:
– Infectious granulomatous disease
(tuberculous or fungal).
– hypersensitivity pneumonia.
– reaction to tumor or drug should be
considered.
55. Summary of diagnostic features of
sarcoidosis on HRCT
• Bronchovascular, interlobular septal and
pleural thickening
• Upper lung predominance common
• Architectural distortion frequent