Non-Infectious Inflammatory Diseases Flashcards
What is sarcoidosis?
a disease involving abnormal collections of inflammatory cells (granulomas) that can form as nodules in multiple organs
Who is usually affected with sarcoidosis? Which gender is more affected?
Young to middle aged black adults with no gender difference
What are the exposures that have been associated with sarcoidosis?
- Insecticides
- Agricultural employment
- Microbial bioaerosols
What are the organs that are most often affected with sarcoidosis?
Involvement of the lymphatic and pulmonary system
What is characteristic about sarcoidosis in the black population, besides more prevalent?
usually more widespread.
What are the components Lofgren’s syndrome? (3) What is it?
Sarcoidosis characterized by:
- Erythema nodosum
- Polyarthralgia
- Bilateral hilar LAD
What is Heerfordt’s syndrome?
a rare manifestation of sarcoidosis. The symptoms include inflammation of the eye (uveitis), swelling of the parotid gland, chronic fever, and in some cases, palsy of the facial nerves
What are the ssx of sarcoidosis?
Like TB, but can include progressive SOB
What are the PE findings with sarcoidosis?
Adenopathy
Skin lesions
What are the lab findings with sarcoidosis?
Hypercalcemia
Elevated LFTs
Increases creatinine
Why should an ECG be obtained with sarcoidosis?
Can lead to conduction abnormalities and arrhythmias
Why should an ophthalmologic exam be performed with sarcoidosis?
Can present with uveitis, uveoparotitis, and retinal inflammation, which may result in loss of visual acuity or blindness
Why should PFTs be obtained with Sarcoidosis?
Most predominant manifestation of sarcoidosis is fibrotic lung disease
Why should you get a TB test with sarcoidosis?
Since so similar ssx, need to r/o
What percent of patients with sarcoidosis have lung involvement?
90%
What percent of patients with sarcoidosis have constitutional symptoms?
30%
What percent of patients with sarcoidosis have palpable LAD?
30%
What percent of patients with sarcoidosis have ski involvement?
25%
What percent of patients with sarcoidosis have ECG changes?
5%
What is lupus pernio?
a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis.
What, generally, is the treatment for sarcoidosis?
Steroids
What are the CBC findings of sarcoidosis?
Leukopenia
True or false: GI and/or renal involvement with sarcoidosis is common
False-both are rare
What are the PFTs like with sarcoidosis?
Restrictive findings
What happens to TLC, VC, RV and DLCO with sarcoidosis?
All decreased
Can you have obstructive lung findings of sarcoidosis?
True
What is stage 0 of the siltzbach radiographic classification for sarcoidosis?
Normal CXR
What is stage I of the siltzbach radiographic classification for sarcoidosis?
Hilar LAD
What is stage II of the siltzbach radiographic classification for sarcoidosis?
Hilar adenopathy
What is stage III of the siltzbach radiographic classification for sarcoidosis?
No adenopathy, abnormal lung parenchyma
What is stage IV of the siltzbach radiographic classification for sarcoidosis?
Fibrotic changes architectural
What are the CT findings of the lungs with sarcoidosis?
Nodular appearance
What comprises the granulomas of sarcoidosis?
Langerhans cells
Macrophages
What is the definitive test for sarcoidosis? What are the findings of this?
BAL–high percentage of lymphocytes (50%), low granulocyte count
What is the CD4/CD8 ratio with sarcoidosis? How sensitive/specific is this?
greater than 3.5
low sensitivity
High specificity
Why should you only biopsy one side of a lung?
May cause pneumothorax
True or false: every patient with sarcoidosis should get some sort of treatment
False
What are the two tests that, when combined, get a high sensitivity/specificity for sarcoidosis?
Endobronchial plus transbronchial biopsies
True or false: most of the time, sarcoidosis is a lifelong disease
False–usually resolves spontaneously
When is the use of systemic steroids indicated for sarcoidosis?
- symptomatic stage II
- stage III
- Any clinical findings present
How long does it take for oral steroids to take effect on sarcoidosis?
3-24 months
In whom is lung involvement with RA more common in: men or women?
Men
What is the usual age of presentation for lung involvement with RA?
50-60 yo
What percent of patients with RA develop ILD?
50%
What are the ssx of ILD secondary to sarcoidosis?
Non-productive cough
DOE
Cubbing
True or false: lung ssx with RA can precede the joint ssx of RA
True
What are the lung exam findings with ILD secondary to RA?
Inspiratory fine crackles
Pleural friction rub
What are the radiographic findings of RA in the lungs?
Cavitary lesions in the lungs
OR
Sponge like lesions
What are the three major drugs to treat RA lung disease?
- Methotrexate
- Etanercept
- Infliximab
What happens with the glucose levels of the interstitial fluid with RA? Why?
Lower, since granulomas there are utilizing it
How common is lung involvement with scleroderma?
75% or patients, and 30% with significant pulmonary ssx
What is the most frequent cause of morbidity and mortality in scleroderma patients?
Lung involvement
What are the ssx of lung involvement with scleroderma?
DOE
What are the two forms of scleroderma involvement of the lungs?
Interstitial lung disease
CREST
What are the PE lung findings with scleroderma?
Inspiratory crackles
Is scleroderma lung involvement usually a restrictive or obstructive disease?
Restrictive with decreased DLCO
What is the major sequelae of CREST syndrome?
pHTN
Decreased DLCO
What are the components of CREST syndrome?
Calcinosis Raynaud's Esophageal dysmotility Sclerodactyly Telangiectasia
What are the usual CT findings with CREST syndrome?
Honeycombing fibrosis
What percent of SLE pts will have lung involvement?
90%
What is the most common presentation of SLE in the lungs?
Serositis
and pleural effusions
What are the consequences of SLE in the lungs?
- Pneumonitis
- Alveolar hemorrhage
- Diaphragmatic dysfunction
What is the major vascular pathology that can happen with SLE, that affects the lungs?
DVT/PE
What are the antibodies that are elevated in Sjogren’s syndrome?
anti-SSA/Ro or anti-SSB/La
What is the most common lung involvement of Sjogren’s syndrome?
Bronchiectasis and bronchiolitis
What type of lung pathology is usually found with Sjogren’s: restrictive or obstructive.Why?
Obstructive d/t bronchiolitis
How do you determine if there is enlarged bronchioles on a CT?
Compare to the arteries
What is the lung involvement with polymyositis/dermatomyositis?
Aspiration pneumonia secondary to diaphragmatic weakness.
What percent of patients with polymyositis/dermatomyositis have lung involvement?
40%
Interstitial fibrosis with polymyositis/dermatomyositis usually involves what part of the lungs?
Lung bases
What is the strongest predictive factor for lung involvement with polymyositis/dermatomy?
The presence of autoantibodies to aminoacyl tRNA synthetase (anti-Jo1)
What is Wegener’s granulomatosis?
Systemic disorder that involves both granulomatosis and polyangiitis. It is a form of vasculitis that affects small- and medium-size vessels in many organs. Damage to the lungs and kidneys can be fatal.
What are the ssx of Wegener’s granulomatosis lung involvement?
Cough
Hemoptysis
Sinus symptoms
What are the antibodies that are elevated with Wegener’s granulomatosis? How sensitive/specific is this?
Antineutrophil cytoplasmic antibody
cANCA
High sensitivity and specificity
What are the urinalysis findings with Wegener’s granulomatosis? (3)
Proteinuria
Hematuria
Red cell casts
What are the usual radiological findings with Wegener’s granulomatosis?
Cavitary lesions
What is Goodpasture syndrome?
a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure. It is thought to attack the alpha-3 subunit of type IV collagen, which has therefore been referred to as Goodpasture’s antigen
What are the ssx of lung involvement with Goodpasture syndrome?
Alveolar hemorrhage
Hemoptysis
What are the antibodies that are diagnostic for Goodpasture syndrome?
Anti-glomerular base membrane antibodies
What are the bronchoscopy findings with Goodpasture syndrome?
Persistent bloody fluid that does not clear on serial samplings
What are the radiological findings of Goodpasture syndrome?
Diffuse Alveolar hemorrhage
What percent of patients with Goodpasture syndrome will not have hemoptysis?
1/3
What is Churg-strauss syndrome?
an autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy)
What is the prodrome phase of Churg-strauss syndrome?
Rhinitis
Sinusitis
Asthma
What is the eosinophilic stage of Churg-strauss syndrome?
Peripheral eosinophilia
Eosinophilic pneumonia
Vasculitis
What age does Churg-strauss syndrome usually present? In which gender is this more common in?
Between 4th and 5th decade
No gender difference
What are the three phases of Churg-strauss syndrome?
Prodrome (asthma ssx)
Eosinophilic
Vasculitis
What are the characteristics of Churg-strauss syndrome lung PFTs?
Reversible obstructive airway ssx
What is the largest cause of mortality with Churg-strauss syndrome?
Cardiovascular compromise
True or false: renal involvement with Churg-strauss syndrome is uncommon
True
What are the CBC values that can be seen with Churg-strauss syndrome?
Eosinophilia
pANCA
Elevated IgE
What are the radiographic findings with Churg-strauss syndrome?
Nonspecific infiltrates