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
On imaging, how does bronchiectasis appear?
Airway diameter is large than blood vessel diameter
What is polymyositis? What is dermatomyositis?
Polymyositis= uncommon inflammatory disease that causes muscle weakness affecting both sides of your body
Dermatomyositis= disease related to polymyositis that affects the muscles and skin
How does polymyositis/ dermatomyositis present with lung involvement?
1) Aspiration pneumonia from pharyngeal muscle weakness
2) Diaphragm elevation and decreased lung volumes
3) Basilar atelectasis
What are the different froms of pulmonary vasculitis?
1) Wegener’s Granulomatosis
2) Churg Strauss Syndrome
3) Goodpasture’s Syndrome
4) Diffuse Alveolar Hemorrhage
What is Wegener’s Granulomatosis?
Necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and kidneys
What is the classic presentation of Wegener’s Granulomatosis?
Middle aged male w/
- Sinusitis or nasopharyngeal ulceration
- Hemoptysis
- Bilateral nodular lung infiltrates
- Hematuria
What serum antibody is associated with disease activity in Wegener’s Granulomatosis? What is the utility of this antibody?
c-ANCA*
*Can be used to differentiate from TB–look v. similar on CXR
What will biopsy of Wegener’s Granulomatosis reveal?
Large necrotizing granulomas with adjacent necrotizing vasculitis
What is Goodpasture Syndrome?
- Rare autoimmune disease
- antibodies attack the basement membrane in lungs and kidneys
- Causes bleeding from the lungs and kidney failure
How can patient’s with Goodpasture Syndrome present?
Alveolar hemorrhage and renal failure
How can you diagnose alveolar hemorrhage?
Bronchoscopy/ alveolar lavage
What is Churg-Strauss Syndrome?
Necrotizing granulomatous inflammation with eosinophils involving the heart and lungs
What is the triad of Churg-Strauss Syndrome?
1) Asthma
2) Hypereosinophilia
3) Necrotizing vasculitis
What antibody correlates with disease progression in Churg-Strauss Syndrome?
p-ANCA
