1. פרופ' נוויל ברקמן מכון הריאה ביה"ח האוניברסיטאי הדסה עין-כרם
Bronchiolitis Obliterans Organising Pneumonia (BOOP), Granulomatosis with Polyangiitis
פרופ' נוויל ברקמן מכון הריאה ביה"ח האוניברסיטאי הדסה עין-כרם

2. Brochiolitis Obliterans and Organising Pneumonia (BOOP)
Clinico- Pathological Diagnosis
Clinical
Radiological
Pathological

3. Brochiolitis Obliterans and Organising Pneumonia (BOOP) Differential Diagnosis
Idiopathic (Cryptogenic Organising Pneumonia)
Infection
Post-transplantation
Collagen vascular disease
Irradiation
Drugs
Other

4. Idiopathic BOOP (Cryptogenic Organising Pneumonia)
M=F, any age (mean 58).
Acute or subacute onset of cough, fever, fatigue, weight loss, dyspnea "slowly or non-resolving pneumonia"
Inspiratory crackles, leukocytosis, raised ESR
X-ray: Bilateral diffuse alveolar infiltrates, peripheral, nodular, lower lobes, pleural effusion is rare.

7. COP- cont.
PFTs: restriction, reduced diffusion, obstruction (20%), hypoxemia.
Diagnosis: characteristic pathology with fibrotic buds in alveoli (organising pneumonia) and bronchiolitis obliterans.
Treatment: Steroids, cyclophosphamide.
2/3 respond, others have progressive disease.

8. Pulmonary Vasculitis Granulomatous vasculitis syndromes
Granulomatosis with polyangiitis (GPA) Wegener's vasculitis
Eosinophilic Granulomatosis with polyangiitis (EGPA) Churg-Strauss vasculitis
Lymphomatoid granulomatosis
Pulmonary-renal syndromes
Microscopic polyangiitis (overlap polyangiitis syndrome)
pauci-immune glomerulonephritis
Classical polyarteritis nodosa
Giant cell arteritis
Takayasu's disease
Behcet's disease

9. Granulomatosis with polyangiitis (GPA) Definition
A multi-organ disease histologically characterized by necrotizing vasculitis involving the small vessels, extensive necrosis & granulomatous inflammation

10. Granulomatosis with polyangiitis Clinical manifestations
Hoffman GS: Ann Intern Med 1992;116:488

14. Granulomatosis with polyangiitis

15. Antineutrophil Cytoplasmic Antibodies (ANCA)

16. Granulomatosis with polyangiitis Pathology
Necrotizing vasculitis: arterioles, venules & capillaries
Granulomatous inflammation
Geographical parenchymal necrosis
Hemorrhagic infarcts
Fibrosis

20. Granulomatosis with polyangiitis Treatment
Mortality >80% within 3 years without adequate treatment
Treatment of choice:
Prednisone 1mg/kg/day
Cyclophosphamide 1-2mg/kg/day (orally)
Remission is achieved in 70-93%

21. Eosinophilic granulomatosis with polyangiitis Churg-Strauss syndrome
History of asthma
Marked blood eosinophilia (up to 10000/ul)
Vasculitis, eosinophilic tissue infiltration
Sinusits
Skin (70%)- nodules,purpura, urticaria
Nervous system- mononeuritis muliplex (66%), CNS
GIT- abdominal pain (60%), diarrhea (33%), bleeding
Cardiac- cardiac failure (50%), pericarditis, hypertension
Renal- dysfunction (50%)
fever, lymphadenopathy

23. Churg-Strauss cont.
Lungs- infiltrates (>70%), usually transient and patchy, also nodules , interstitial infiltrates, pleural effusion (1/3).
Obstructive PFTs, elevated IgE, anemia, elevated ESR
BAL- eosinophilia (33%), eosinophils in pleural fluid
Biopsy- necrotising giant cell vasculitis (small arteries and veins), eosinophils, granulomas.
Treatment: Steroids, cyclophosphamide.

24. Rheumatoid Arthritis Pulmonary manifestations
Pleural disease
pleurisy (20%), effusion (4%), unilateral (80%), R>L
middle-aged male, any time during the illness
Associated with nodules not arthritis
Fluid: exudate, LDH>1000, low glucose (<50mg% in 80%),low pH, lymphocytic, RF +, low complement

25. RA-cont. Interstitial pneumonitis
identical to idiopathic pulmonary fibrosis
fullblown in 2%, abnormal PFTs in 41%
M>F, patients are RF positive
Cough, dyspnea, dry rales, clubbing
hypoxemia, restrictive PFTs, reduced DCO
Variable response to steroids
Poor prognosis (survival 3-5 years)
May be drug-induced (methotrexate, gold, penicillamine)

26. RA-cont.
Nodules:
single (1/3) or multiple (2/3), more common in men
related to disease activity and skin nodules
asymptomatic, may cavitate, rarely hemoptysis
peripheral, 0.3-7cm diameter
may respond to steroids
Caplan's syndrome: pulmonary nodules with RA in coal miners (coal miner's pneumoconiosis)

27. RA-cont. Bronchiolitis Obliterans Pulmonary hypertension
airways obstruction, 60% of smokers, 30% of non-smokers
peribronchial inflammatory infiltrate
may be drug-induced
Pulmonary hypertension
pulmonary arteritis

28. Systemic Lupus Erythematosis Pulmonary manifestations
Pleura:- pleuritis or effusion, 50-75% of patients, presenting symptom in 1/3, fluid is an exudate, PMNs, raised ANF, LE cells
Interstitial pneumonitis:- acute (fever,cough, progressive hypoxemia) or chronic
Pulmonary thromboembolic disease:- lupus anticoagulant positive patients
Diaphragmatic dysfunction
Atalectasis:- subsegmental, bibasilar
INFECTION!

29. Goodpasture's syndrome
Alveolar hemorrhage
Glomerulonephritis
Anti-glomerular basement membrane antibody

30. Goodpasture's syndrome
Young males
Autoimmune disorder
Clinical features:
Rapidly-progressive glomerulonephritis
Hematuria, proteinuria, renal failure
Hemoptysis, dyspnea, cough
Anemia
Arthralgia

31. Goodpasture's syndrome
Laboratory features:
X-ray: transient infiltrates
Urine:red cells, casts
Iron-deficiency anemia
Restrictive defect on lung-function tests with increased diffusion capacity.
Diagnosis:
Hemosiderin-laden macrophages
Anti-GBM antibodies
Renal biopsy
Treatment:
Plasmapharesis, steroids, cyclophosphamide.