EXAM #2: NON-INFECTIOUS INFLAMMATORY LUNG DISEASE Flashcards
What is Sarcoidosis?
- Mutlisystem granulomatous inflammatory disease
- Unknown etiology
What patient population is commonly affected by Sarcoidosis?
Aoki= young-middle aged adults (3rd decade)
Pathoma= African American females
How does the presentation of Sarcoidosis in African Americans differ from other patients?
African Americans= more severe and more widely distributed
What is the most common presenting symptom of Sarcoidosis?
Dyspnea with nonproductive cough
*Less common sx. include: constitutional sxx, lymphadenopathy, skin changes, dysrhythmia
What organs/ organ systems are commonly involved outside of the lungs?
1) Lymphatic system
2) Uvea= uveitis
3) Skin= cutaneous nodules, lupus perino*, or erythema nodosum
4) Salivary or lacrimal glands (Resembles Sjogren’s Syndrome)
*violet rash involving the nose and cheeks.
If a patient presents with a history and physical suspicious for Sarcoidosis, what should be included in the diagnostic work-up?
1) PFT
2) Labs
- Ca++
- ACE
- Liver enzymes
- Creatinine
3) ECG
4) Ophthalmalogic exam (uveitis)
5) TB skin test (r/o TB)
What type of presentation is associated with Sarcoidosis on PFT? Draw the flow-volume loop that you would expect to see compared to the normal.
Restrictive
- Decreased TLC
- Decreased VC
- Decreased RV
*Note that this patient CAN present with obstructive disease depending on the location of the granulomas
Outline the radiographic staging of Sarcoidosis.
0= Normal I= Hilar adenopathy II= Hilar adenopathy + abnormal lung parenchyma III= No adenopathy BUT abnormal lung parenchyma IV= Fibrotic changes/ structural distorsion
Describe the appearance of Sarcoidosis on CT.
Granulomatous nodules
If history, exam, CXR, and/or CT point to Sarcoidosis, how do you confirm diagnosis?
Brochoscopy
What will happen with most patients affected by Sarcoidosis?
Spontaneous remission NOT requiring treatment
What are the indications for systemic steroids in Sarcoidosis?
1) Cardiac involvement
2) Ocular disease
3) Neurologic disease
4) Hypercalcemia
5) Lupus pernio
6) Symptomatic stage II
7) Stage III
List the most common connective tissue disorders that involve the lung.
1) RA
2) Scleroderma
3) SLE
4) Sjogren Syndrome
5) Dermatomyositis and polymyositis
What is the common age of patients with Rheumatic Lung Disease? Is there a gender predominance?
- 50-60 y/o
- Yes, males
What are the signs/sx. of Rheumatic Lung Disease (RLD)?
1) Non-productive cough
2) Dyspnea on exertion
3) Clubbing
4) Inspiratory fine crackles
5) Pleural rub
Note that Rheumatic Lung Disease may PRECEED joint disease
How does RLD appear on imaging?
1) Nodules that can CAVITATE
2) Interstitial disease
*Note that TB and other infections can cavitate and must be ruled out first before starting on immunosuppressive therapy
What are the treatments for RLD?
1) Methotrexate
2) Etanercept
3) Infliximab
Pleural effusion associated with RLD has characteristic lab finding?
Low glucose
What is Scleroderma? What are the two presentations of Scleroderma?
- Generally, an autoimmune disorder characterized by sclerosis of the skin and visceral organs. There is:
1) Diffuse Scleroderma
2) CREST Syndrome
C= calcinosis R= raynaud E= esophageal dysmotility S= sclerodactyly T= telangiectasias
What is CREST Syndrome associated with?
Pulmonary HTN*
*This is an indication for vasodilators
What is the presentation of diffuse Scleroderma?
- Inspiratory crackles
- Restrictive lung physiology
What is the most common presentation of SLE involving the lung?
Serositis i.e. inflammation of the serous tissues of the body, such as the pleura
Aside from serositis, what else is associated with SLE involving the lung?
1) Pneumonitis
2) Alveolar hemorrhage
3) Diaphragmatic dysfunction
4) Increased risk for DVT/PE*
*Emphasized in lecture
How does Sjogren’s Syndrome present when it involves the lung?
- Bronchiectasis
- Bronchiolitis
*Especially in the context of other Sjogern’s Symptoms i.e. dry eyes, dry mouth