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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5537_Библиотеки_им_академика_М_И_Перельмана.pdf
History of exposure to asbestos at shipyards, while working with car
brakes and installing insulation, to silica at construction sites or as a sandblaster, to coal dust among miners, to heavy metals in metal workers, and
to organic/inorganic substances in farmers are just some examples of
work-related exposures that can cause significant lung disease. It should
be noted here that a typical history of hypersensitivity pneumonitis
(immune reaction to organic dust) includes worsening of symptoms while
at work or when repetitively exposed to a certain hobbies or locations.
Similar symptoms are seen in occupational asthma, and this diagnosis
should be considered as part of the differential diagnosis.
Patients can have indirect exposures as well, as exemplified by a land-
mark paper in 1979 by Selikoff’s group, who found that 35% of household contacts of asbestos workers had radiographic evidence of asbestos
exposure despite there being no direct exposure to the substance.
4
It was
theorized that home contamination from shoes and work clothes was the
source of asbestos exposure. Also, physicians must be wary that patients
may not acknowledge their exposures or be unaware of inciting agents.
For example, bird fanciers may not offer exposure history due to fear of
being separated from their pets.
Special testing may ultimately be warranted to confirm occupational
exposure (such as demonstration of ferruginous bodies in lung biopsis of
asbestosis or lung biopsies or a hypersensitivity panel for serologic
evidence of immune reaction to organic antigens).
5,6
Particular attention to extrapulmonary signs and symptoms is also
important to the diagnosis of patients with occult rheumatologic disease as
a cause of ILD. Many patients who are referred to advanced lung disease
programs for idiopathic lung disease are found to have an underlying systemic rheumatologic disease (see Fig. 2).
7
Clinicians should elicit signs and
symptoms suggestive of rheumatoid arthritis, scleroderma, systemic lupus
erythematosis, dermatomyositis, polymyositis, Sjogren’s disease, sarcoidosis, or “overlapping” rheumatologic syndromes and pursue confirmatory
testing with the guidance of rheumatology specialists (see Table 2).
8–17
In addition, up to 10% of ILD patients meet criteria for “undifferenti-
ated connective disease” (UCTD) but do not meet classically defined
324
J. Kim and T. J. Harkin
criteria for a known rheumatologic illness. Recent data suggest that many
patients with nonspecific interstitial pneumonia (NSIP) also meet criteria
for UCTD.
22,23
Similarly, patients with sarcoidosis, lymphangioleiomyomatosis, or pulmonary Langerhans cell histiocytosis (LCH) have unique clinical and
radiographic features (see Table 2 and Figs. 3–5).
Idiopathic interstitial pneumonias are a distinct group of DPLDs of
unknown etiology that requires a considerable amount of expertise to
define and diagnose. However, it is critical to recognize the presentation
of idiopathic pulmonary fibrosis (IPF), because it is the most common and
most serious idiopathic interstitial pneumonia. IPF is found most commonly in males 40–70 years of age who have a previous smoking history
and may have a familial clustering of pulmonary fibrosis. There is an
important protypical clinical–radiographic presentation of this entity that
allows the confirmation of this diagnosis without the need for a surgical
325
Evaluation and Treatment of Diffuse Parenchymal Lung Disease
Fig. 2. Scleroderma-related DPLD: A woman with a history of Raynaud’s phenomenon,
dysphagia, and skin features suggestive of scleroderma. Chest CT demonstrated
parenchymal disease typical of NSIP, an enlarged pulmonary artery (pulmonary hypertension), and an enlarged, dilated food-filled esophagus (arrow) that was later studied by
esophagram.
326
J. Kim and T. J. Harkin
p
Table 2. Clinical Features and Tests that Aid in Differentiation of DPLD from Systemic Diseases
9–20
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
Rheumatoid 4 of 7 criteria Pleural involvement Hand x-ray Antinuclear antibody
arthritis Morning stiffness >1 hr Pericardial involvement (erosions) Rheumatoid factor
ACR (1987) Arthritis >3 joints Bronchiectasis and Anti-CCP
criteria vs. Hand arthritis airways disease
CCP-7-replace Symmetric arthritis Interstitial lung disease
rheumatoid Rheumatoid nodules UIP or NSIP pattern
nodules for (vs. Positive CCP) Small nodules
early RF RF+ Necrobiotic lung nodules
Radiographic changes
Scleroderma Limited SS sine scleroderma Interstitial lung disease, Nailfold Antinuclear antibodies
3 major categories Raynaud’s phenomena UIP or NSIP pattern capillaroscopy with a nucleolar
Limited SS (RP) objective documentation Pulmonary artery Swallowing staining pattern
sine scleroderma or RP by history + nailfold enlargement (Pulm studies (90% of SSc
Limited cutaneous capillaroscopy (dilation, HTN with mosaicism Echocardiogram patients)
SS (CREST) avascular areas) and and centrilobular nodules) Anticentromere
Diffuse cutaneous positive serology Dilated esophagus (ACA), antitopoiso
merase-I (Scl-70),
(LeRoy, 1988) Limited cutaneous SS anti-RNA poly
Above plus skin tautness of merase, U3-RNP
fingers, hands, forearms, antibodies
legs, feet, toes, neck, and face
(Continued)
327
Evaluation and Treatment of Diffuse Parenchymal Lung Disease
p
Table 2. (Continued )
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
CREST calcinosis,
Raynaud’s phenomenon,
esophageal hypomotility,
sclerodactyly, telangectasia
Diffuse cutaneous SS
RP within 1 year of onset of skin
changes (puffy or hidebound)
Truncal and acral skin involvement
Presence of tendon friction rubs
Interstitial lung disease, oliguric
renal failure, diffuse
gastrointestinal disease, and
myocardial involvement
Absence of ACA
Nailfold capillary dilatation and
capillary destruction
Antitopoisomerase antibodies
(30% of patients)
Sjogren’s disease Primary SS: 4 of the 6 items Lung cysts (LIP) “Keratoconjunctivitis Antinuclear
below (I–IV) if at least IV Interstitial fibrosis, sicca tests” antibody
(Continued )
328
J. Kim and T. J. Harkin
p
Table 2. (Continued )
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
(histopathology) or VI honeycombing and Schirmer’s I test, Antibodies to
(serology) is positive pulmonary fibrosis performed without Ro(SSA) or La(SSB)
Or 3 of the 4 objective Small airways disease anesthesia (<5 mm antigens, or both
criteria items (items III–VI) Risk of in 5 min)
Subjective pseudolymphoma Rose bengal score or Cryoglobulinemia
I. Ocular symptoms (dry eyes and lymphoma other ocular dye Hypocomplementemia
>3 months; recurrent sensation of score (>4 according IgG4-positive
sand or gravel in the eyes, tear to van Bijsterveld’s lymphoproliferative
substitutes more than scoring system) syndrome
3 times a day)
II. Oral symptoms: dry mouth Unstimulated whole
>3 months, persistently swollen salivary flow
salivary glands; frequently (<1.5 ml in 15 min)
drink liquids to aid in
swallowing dry food
Objective Parotid sialography
III. Ocular signs — positive showing the
result for at least one of the presence of diffuse
following two tests: sialectasias (punctate,
1. Schirmer’s I test cavitary or
2. Rose bengal score
(Continued)
329
Evaluation and Treatment of Diffuse Parenchymal Lung Disease
p
Table 2. (Continued )
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
IV. Histopathology: in minor destructive pattern),
salivary glands without evidence of
V. Salivary gland involvement: obstruction in the
positive result for at least one major ducts
of the following diagnostic tests: Salivary scintigraphy
1. Unstimulated whole showing delayed
salivary flow uptake, reduced
2. Parotid sialography concentration and/
3. Salivary scintigraphy or delayed
VI. Antibodies to Ro(SSA) or excretion of tracer
La(SSB) antigens, or both Possible gland biopsy
Mixed Connective Mixed connective tissue Interstitial lung disease Echocardiogram Antinuclear antibody
Tissue Disease (overlap syndrome): Pulmonary artery Anti-RNP
Combination of SLE, enlargement (Pulm antibodies titer
scleroderma or PSS, and HTN) with mosaicism (an RNAsepolymyositis–dermatomyositis and centrilobular sensitive extractable
nodules >1:1600)
Positive ANA
(commonly
>1:1000 and often
greater than
1:10,000) speckled
(Continued )
330
J. Kim and T. J. Harkin
p
Table 2. (Continued )
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
Systemic Lupus 4 or more of 11 criteria Pleural disease
Erythematosis 1. Malar rash Pericardial disease
Diagnostic and 2. Discoid rash Inflammatory pneumonitis Plain radiographs Antinuclear antibodies
Therapeutic 3. Photosensitivity (diffuse infiltrates/ of involved (ANA), ANA is
Criteria 4. Oral or nasopharyngeal consolidation/effusions joints positive in
Committee of ulceration with DAD on path) Renal significant titer
the ACR, 1997 5. Arthritis (nonerosive arthritis Diffuse alveolar ultrasonography (usually 1:160 or
2 peripheral joints — hemorrhage (DAH) to assess kidney higher) in virtually
tenderness, swelling, or neutrophilic capillaritis; disease all patients with
effusion) rapid resolution of Urine protein SLE
6. Serositis (pleuritis, pericarditis) findings Urine sediment Antinuclear Ab
7. Renal disorder (persistent Interstitial pulmonary Echocardiography Antiphospholipid
proteinuria or cellular casts) fibrosis (not common) (pericardial antibodies
8. Neurologic disorder Pulmonary hypertension disease and Antibodies to double(seizure psychosis) Diaphragmatic dysfunction evidence of PE) stranded DNA
9. Hematologic disorder (shrinking lung Computed Anti-Smith (Sm)
anemia, leukopenia, syndrome; high tomography antibodies
(hemolytic lymphopenia, diaphragms); (CT) (e.g. for Measurement of serum
thrombocytopenia with Pulmonary emboli with abdominal pain, complement levels
absence of offending drug) infarction suspected C3 and C4
10. Immunologic disorder pancreatitis)
(anti-DNA ab, anti-Sm ab,
antiphospholipid antibodies)
(Continued)
331
Evaluation and Treatment of Diffuse Parenchymal Lung Disease
p
Table 2. (Continued )
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
11. Antinuclear antibody in the Magnetic resonance
absence of drugs known to imaging
be associated with ‘‘drug- (neurologic
induced lupus’’ syndrome involvement)
Contrast
angiography
for vasculitis,
medium-sized
arteries
Dermatomyositis/ Symmetric proximal muscle
PM weakness
Typical rash of DM (only Aspiration PNA EMG CPK
distinguishing clinical Interstitial lung Muscle biopsy Aldolase
feature between DM disease UIP or “Myositis panel”:
and PM) ex. NSIP pattern auto-Abs to
heliotrope rash Consolidations synthetases
Elevated serum muscle (COP) encompassed anti-
enzymes Jo-1, OJ, EJ, KS,
Myopathic changes on PL7, and PL12
electromyography specificities
Characteristic muscle Systemic
biopsy abnormalities sclerosis-specific
(Continued )
332
J. Kim and T. J. Harkin
p
Table 2. (Continued )
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
and absence of aAbs (aAbs to
histopathologic signs of centromeres,
other myopathies topo I,Th, and
RNA polymerases
I/III) for overlap
syndromes
Sarcoidosis Diagnosis: Noncaseating Pulmonary Sarcoidosis Kveim–Siltzbach ACE level is an
granuloma in biopsy of two Chest x-ray pattern test intradermally insensitive and
different organs (with all other 1. Stage 1: hilar biopsy 4 wk nonspecific test
causes of granulomas excluded) adenopathy FDG-PET or cardiac
excluded or a positive 2. Stage 2: hilar MRI for
Kveim test or Lofgren’s adenopathy with sarcoidosis of
syndrome (no biopsy needed) reticulonodular the heart
Other signs and symptoms infiltrates MRI of the brain/
(organs affected) 3. Stage 3: reticulonodular spine if
Constitutional symptoms infiltrates concerned for
Lacrimal gland enlargement 4. Fibrocystic lung disease neurosarcoidosis
Skin findings: erythemanodosum,
lupus pernio
(Continued)
333
Evaluation and Treatment of Diffuse Parenchymal Lung Disease
p
Table 2. (Continued )
Disease Specific Clinical
Features and Summary Radiographic Spectrum Potential Clinical Potential Laboratory
Potential Etiology of Diagnostic Criteria in the Lung Testing Data
Lymph node enlargement
Cardiac symptoms including
palpitations
Neurologic symptoms, including
uveitis and facial palsies
Hepatosplenomegaly
Liver disease
Renal stones /renal disease
Symptoms of hypercalcemia
Lymphangioleio- Definite LAM Multiple thin-walled round Abdominal CT vs. MRI
myomatosis 1. Characteristic or compatible well-defined air-filled for detection of
(LAM) lung HRCT, and lung biopsy cysts angiomyolipomas,
(Johnson fitting the pathologic criteria lymphangioleiomyomas,
McCormack) for LAM or lymphadenopathy
2. Characteristic lung HRCT and Renal mass biopsy
any of the following: Lung biopsy LAM cells
a. Renal angiomyolipoma (smooth muscle cells)
(kidney biopsy), thoracic or expressing markers
abdominal chylous effusion, of smooth muscle
(Continued)