1. ISCEE Respiratory System
23rd Nov 2010

2. CASE PRESENTATION 1
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.

3. Examination
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
Musculoskeletal system: no arthritis and no muscle weakness
CNS: NAD
No cranial nerve palsy, no peripheral neuropathy

4. Investigation 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)

6. SARCOIDOSIS
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

7. 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.

8. Treatment
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 6-month course is adequate for pulmonary disease.

9. EPIDEMIOLOGY Sarcoidosis affects men and women of all races and ages
Usually presents in adult younger than 40 years more frequent between 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.

10. Case 2

11. Case Presentation 2
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. 

12. Overview Major catagories of pulmonary disease associated with RA:
Pleural effusion
Nodular lung disease
Diffuse interstitial fibrosis
BOOP (bronchiolitis obliterans organizing pneumonia)
Pulmonary vasculitis
Alveolar hemmorhage
Obstructive disease
Infections

13. Rheumatoid Arthritis And The Lung
Broad differential for pleuropulmonary disease in those with rheumatologic disorders:
Secondary to, or associated with the underlying rheumatic disease
Secondary to immunosuppression (infection)
Secondary to drug therapy
Coexistant medical problems
Overlap syndromes

14. Pleural Effusion Most common pulmonary manifestation of RA
Often incidental finding on CXR
Patients often asymptomatic
?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

15. Pleural Effusion Treatment: Probably best to control the underlying RA
None needed if asymptomatic
Repeated thoracentesis or pleurodesis
NSAIDs, steroids
Intrapleural steroids
Probably best to control the underlying RA

16. Nodular Lung Disease
Pulmonary nodules in RA first described by Caplan in 1953
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

17. Nodular Lung Disease 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

18. Nodular Lung Disease Management  usually observation suffices
Transbronchial biopsy or transthoracic needle aspiration to rule out malignancy or other pathologic process

19. Diffuse Interstitial Fibrosis
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

20. Diffuse Interstitial Fibrosis
Prognosis is poor
Treatment usually includes corticosteroids, azathioprine or other immunomodulating medications (e.g., cyclophosphamide)
?Single lung tranplantation
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)

21. Infections Persistent problem in those with RA
Many confounding factors, especially corticosteroid or immunosuppressive medication
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

22. Drug Related Pulmonary Disease
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