ISCEE Respiratory
System
23rd Nov 2010
CASE PRESENTATION 1
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Mrs R, 44 years old lady with no previous medical or surgical
history
Main complaint was painful red eye for one year associated with
discomfort, photophobia and blurred vision.
Seen by ophthalmologist and diagnosed with bilateral chronic
uveitis
She was then investigated to determine aetiology
She has a history of Bells Palsy which resolved spontaneously
after one week and a history of hyper-pigmented scaly lesion on
forehead.
There is no history of cough or dyspnoea.
No history suggestive of TB
No history of joint pain.
Examination
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On examination she was a well looking young, not dyspnoeic,
 BP 129/88
 PR 93/minute
 No significant lymphadenopathy
 Hyper pigmented lesions on the forehead
 Chest clear
 CVS :NAD
 Abdomen no organomegaly
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Musculoskeletal system: no arthritis and no muscle weakness
CNS: NAD
No cranial nerve palsy, no peripheral neuropathy
Investigation
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FBC: Normal
U&E: Normal
LFT: Normal
CALCIUM LEVEL: Slightly raised
ESR: 40mm per hour
ANF: Negative
SACE LEVEL: 111 (Normal less than 52)
LUNG FUNCTION TEST: Normal
CHEST X RAY (next slide)
SARCOIDOSIS
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Multisystem inflammatory disease of unknown
etiology that predominantly affects the lungs.
Manifested by the presence of non-caseating
granulomas (NCGs) that may affect any organ
system
The many forms and presentation of this disease
and the lack of a single diagnostic test can make
the diagnosis challenging
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The lungs are involved in more than 90 percent
of patients, with sarcoid usually presenting as
interstitial disease.
Symptoms are dry cough, dyspnea, and chest
discomfort.
Pulmonary sarcoidosis has an unpredictable
course that may result in spontaneous
remission or lead to progressive loss of lung
function with fibrosis.
Airway involvement can occur and may result
in airflow limitation, persistent cough and, in
severe cases, bronchiectasis.
Treatment
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The majority of patients will have spontaneous remission and a
generally benign clinical course.
Treatment is reserved for
 patients with worsening pulmonary function tests
 patients with worsening pulmonary symptoms (cough,
shortness of breath, chest pain or hemoptysis) and
 patients with extra pulmonary sarcoidosis including arthritis,
neuropathy, cardiac and renal sarcoid, also in,
 patient with intractable fatigue, weakness or fever.
Corticosteroids are the mainstay of therapy.
 Generally, prednisone given daily and then tapered over a 6month course is adequate for pulmonary disease.
EPIDEMIOLOGY
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Sarcoidosis affects men and women of all races and ages
Usually presents in adult younger than 40 years more frequent
between 20 - 29 years, and slightly more predominant in women
than in men.
Course of sarcoidosis is variable, ranging from self limited acute
disease to a chronic debilatating disease.
Spontaneous remissions occur in nearly two thirds of patients.
CASE 2
Case Presentation 2
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63 years old female presented with gradual onset SOB over the
last 6 months.
She is known to suffer from Rheumatoid arthritis for the last 3
years and has been on treatment with methotrexate for the same.
She has previously had an episode of pleural effusion which was
drained and investigated but was found to be an exudate with
high protein and low glucose.
On examination she had dull percussion note and absent air
entry in her left lower zone of the chest.
Patient’s bloods were within normal limits.
Chest x ray confirmed a pleural effusion.
Overview
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Major catagories of pulmonary disease associated with
RA:
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Pleural effusion
Nodular lung disease
Diffuse interstitial fibrosis
BOOP (bronchiolitis obliterans organizing pneumonia)
Pulmonary vasculitis
Alveolar hemmorhage
Obstructive disease
Infections
Rheumatoid Arthritis
And The Lung
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Broad differential for pleuropulmonary disease in those
with rheumatologic disorders:
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Secondary to, or associated with the underlying rheumatic
disease
Secondary to immunosuppression (infection)
Secondary to drug therapy
Coexistant medical problems
Overlap syndromes
Pleural Effusion
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Most common pulmonary manifestation of RA
Often incidental finding on CXR
Patients often asymptomatic
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?Reduced physical activity prevents symptoms
Most common symptoms: pleuritic pain, dyspnea, cough
Pleural effusions can precede or occur simultaneously with joint
symptoms in 25%
More common in men with high RF titer and active arthritis
Can be uni- or bilateral, resolve, recur or persist for months
Post-mortem studies  almost 50% of patients with RA have
pleural effusions
Pleural Effusion
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Treatment:
None needed if asymptomatic
 Repeated thoracentesis or pleurodesis
 NSAIDs, steroids
 Intrapleural steroids
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Probably best to control the underlying RA
Nodular Lung Disease
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Pulmonary nodules in RA first described by Caplan in
1953
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Discovered multiple bilateral nodules on CXR of coal miners
with RA
 Caplan’s syndrome: Pneumoconiosis in RA patient leading
to multiple basilar pulmonary nodules and mild airflow
obstruction
Only pulmonary manifestation specific for RA
Can occur before, with or after the joint manifestations of
RA
Usually asymptomatic, but can cause coughing and rarely
hemoptysis
Nodular Lung Disease
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Usually multiple, bilateral nodules
Range from few millimeters to several centimeters in size
Typically occur just below the pleura or associated with
interlobular septa
Can lead to bronchopleural fistula, pneumothorax, abcess
or cavition
Can remain static, resolve, increase in size or undergo
malignant transformation
More common in men, ?association with smoking
Nodular Lung Disease
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Management  usually observation suffices
Transbronchial biopsy or transthoracic needle
aspiration to rule out malignancy or other
pathologic process
Diffuse Interstitial Fibrosis
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More common in those with severe RA
Most modifiable risk factor: smoking
 >25 pack-year smoking history significantly more likely to
have radiographic evidence of ILD
Usually occurs about five years after joint symptoms present, but
can predate them
Occurs mostly in those with subcutaneous nodules and high RF
Symptoms: progressive SOBOE and productive cough most
common
 Also: increased RR, clubbing, crepitations at lung bases,
pulmonary hypertension
CXR  Reticulated pattern with progression to fine nodularity
and honeycombing
Diffuse Interstitial Fibrosis
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Prognosis is poor
Treatment usually includes corticosteroids, azathioprine
or other immunomodulating medications (e.g.,
cyclophosphamide)
?Single lung tranplantation
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Usually better results in those with RA associated interstitial
fibrosis
Usually too many comorbidities for transplantation surgery
(e.g., osteoporosis, decreased mobility)
Newer therapies (e.g., TNF blockers)
Infections
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Persistent problem in those with RA
Many confounding factors, especially corticosteroid or
immunosuppressive medication
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May mask the signs of infection
Lymphocyte abnormalities in RA?
Patients with RA have greater occurrence of bronchitis,
bronchiectasis and pneumonia than controls with
degenerative joint disease
Drug Related Pulmonary Disease
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Methotrexate:
Presents with dyspnea, cough and fever
 Usually subacute
 50% of cases diagnosed within 32 weeks of initiating
MTX
 Re-challenge with MTX causes high rate of
recurrence of lung injury
 17% of patients who develop lung disease due to
MTX will die of this complication
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