Pulmonary III - Restrictive Lung Disease and Diffuse Lung Syndromes Flashcards
Sarcoidosis is a disease of …
- -diagnosis is made by …
- -describe the lymphadenopathy
- -who is predisposed?
disease of unknown cause characterized by noncaseating granulomas in many tissues & organs
DIAGNOSIS MADE BY EXCLUSION
bilateral hilar lymphadenopathy or lung involvement is visible on radiographs in 90%
F>M; 10X greater in Blacks than Whites; highest in southeastern United States
Pathogenesis of sarcoidosis
- -what is the cause?
- -what cells are seen?
cause unknown; granulomatous tissue suggests persistent, poorly degradable antigen
- -increased CD4 lymphocytes, activated macrophages; cytokine secretion
- -influx of monocytes, alveolitis, noncaseating granulomas progressing to fibrosis
- -T-cell proliferation oligoclonal, but no specific organism identified
Morphology
noncaseating granulomas in all involved tissues
aggregates of tightly clustered epithelioid cells,
often w/ Langhans’ or foreign body giant cells
enclosed by fibrous rims; may be replaced by fibrous scar
laminated concretions compose of calcium and proteins known as Schaumann’s bodies
stellate inclusions known as asteroid bodies enclosed within giant cells (60%)
What organs are involved in sarcoidosis
Lungs Lymph nodes Spleen LIver Bone marrow Skin Eye, its associated glands, and the salivary glands
Lungs WRT to sarcoidosis
grossly no demonstrable alteration; coalescencing granulomas may produce small nodules
- -distributed along lymphatics around bronchi, blood vessels
- -relative frequency of submucosal lesions
- -lesions in lungs tend to heal; fibrosis and hyalinization
Lymph nodes WRT to sarcoidosis
involved in almost all cases; hilar and mediastinal nodes; enlarged, discrete, and sometimes calcified
Spleen WRT to sarcoidosis
75% cases, but enlarged in only 18%; barely visible nodules
Liver WRT to sarcoidosis
moderately enlarged with scattered granulomas in portal triads
Bone marrow WRT to sarcoidosis
phalangeal bones of hands/feet; sm. circumscribed areas of bone resorption
Skin WRT to sarcoidosis
30-50% of cases; discrete subcut. nodules, erythematous plaques, or flat lesions
Eye, its associated glands, and the salivary glands WRT to sarcoidosis
1/5; iritis, corneal opacities; bilateral sarcoidosis of the parotid (uveoparotid involvement - Mikulicz’s syndrome
Clinical course of sarcoidosis
aried symptomology; may be found unexpectedly on chest films as bilateral hilar adenopathy
may have peripheral lymphadenopathy, cutaneous lesions, eye involvement
insidious onset of respiratory abnormalities (SOB, cough, chest pain, hemoptysis)
constitutional signs (fever, fatigue, weight loss, anorexia, night sweats)
unpredictable course; may be progressive or intermittent
may resolve spontaneously or with steroid therapy
Sarcoidosis
- -recovery?
- -permanent damage?
- -death?
65-70% recover with minimal or no residual manifestations
20% have some permanent loss of lung function
10% dies of cardiac or CNS damage
Sarcoidosis staging
stage I - hilar lymphadenopathy alone
stage II - adenopathy and pulmonary infiltrates
stage III - pulmonary disease and NO adenopathy - few spontaneous remissions and most likely to develop chronic pulmonary fibrosis
Idiopathic pulmonary fibrosis
- -general features
- -advanced cases
- -progression? death?
idiopathic diffuse interstitial inflammation and fibrosis
general features - similar in presentation to other interstitial fibrotic processes; about 50% idiopathic
advanced cases - respiratory difficulty, hypoxemia and cyanosis with 2° pulmonary hypertension
some very rapid (weeks), most cases death occurs in about 2 years
Pathogenesis of idiopathic pulmonary fibrosis
–inflammation response and how it effects the lung
stereotyped inflammatory response of the alveolar wall to injuries
alveolar wall injury –> interstitial edema and accumulation of inflammatory cells –> fibroblasts proliferate –> progressive fibrosis of interalveolar septa and intra-alveolar exudate
progressive fibrosis of interalveolar septa and intra- alveolar exudate
?immune mechanisms; high levels of circulating immune complexes or cryoimmunoglobulins; granular deposits of IgG
Morphology of idiopathic pulmonary fibrosis
- -early
- -late
- -early: pulmonaryedema,intra-alveolar exudation, hyaline membranes, septal infiltrates
- -hyperplasia of type II pneumocytes; cuboidal or even columnar cells lining alveolar spaces
- -fibrous organization of the intra-alveolar exudate/ thickening of interstitial septa
- -honeycomb-lung with small cysts and intimal thickening
late: lungs become solid with alternating areas of fibrosis and normal lung
Rheumatoid arthritis
- -chronic pleuritis
- -diffuse interstitial pneumonitis with fibrosis
- -intrapulmonary rheumatoid nodules
- -rheumatoid nodules with pneumoconiosis (Caplan’s syndrome)
- -pulmonary hypertension
What are some diffuse pulmonary hemorrhage syndromes?
Goodpasture’s Syndrome
Wegener’s granulomatosus
Idiopathic Pulmonary Hemosiderosis
Goodpasture’s Syndrome
- -what is this?
- -when does is present? who in?
- -what is the pathology like?
- -triggering event?
rare; rapidly progressive glomerulonephritis, and a necrotizing hemorrhagic interstitial pneumonitis
2nd-3rd decade; M>F
- -antibodies against antigens in glomerular and pulmonary basement membranes
- -linear deposits of immunoglobulin by immunoflourescence
- -plasma exchange to remove anti-basement membrane antibodies and mediators of inflammation
triggering event unknown: ?toxic pulmonary injury, viral infection, exposure to solvents, smoking
Goodpasture’s
- -what is the presentation like?
- -what does the lung look like pathologically?
- -which symptoms come first?
hemoptysis and focal pulmonary consolidations
intra-alveolar hemorrhages, fibrous thickening of septa, hypertrophy of septal cells
areas of red-brown consolidation
respiratory symptoms precede renal glomerulonephritis; uremia cause of death
Wegener’s granulomatosis
- -when does it present?
- -what are the pathologic presentation?
5th decade; progressive, fatal disease
vascular necrotizing granulomas of upper and lower respiratory tract
- -focal necrotizing vasculitis affecting small to medium-sized vessels
- -persistent pneumonitis with bilateral nodular and cavitary infiltrates
- -chronic sinusitis, ulcerations of the nasopharynx, epistaxis
Wegener’s granulomatosis
- -inflammatory cells?
- -associated with what AB?
- -what other kind of disease is there?
granulomatous vasculitis with extensive fibrosis, atelectasis
–perivascular granulomatous inflammation with giant cells
associated with c-ANCA (anti-neutrophil cytoplasmic antibodies)
some have renal disease with focal or diffuse necrotizing glomerulitis
Idiopathic pulmonary hemosiderosis
–hemorrhage into alveolar spaces with resulting
interstitial fibrosis and hemosiderosis
–occurs in children and younger adults
–similar to Goodpasture’s in presentation, but NO anti-basement membrane antibodies
–productive cough, hemoptysis, anemia, weight loss and diffuse pulmonary infiltrations
