מנחים: פרופ’ דן אדם. מהנדס יוחנן ארז. ביצוע: גיא אגוזי, מוטי שיאון.
Bronchiolitis Obliterans Organising Pneumonia (BOOP), Granulomatosis with Polyangiitis פרופ '...
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Transcript of Bronchiolitis Obliterans Organising Pneumonia (BOOP), Granulomatosis with Polyangiitis פרופ '...
Bronchiolitis Obliterans Organising Pneumonia (BOOP), Granulomatosis with Polyangiitis
פרופ' נוויל ברקמןפרופ' נוויל ברקמןמכון הריאהמכון הריאה
ביה"ח האוניברסיטאי הדסה עין-כרםביה"ח האוניברסיטאי הדסה עין-כרם
Brochiolitis Obliterans and Organising Pneumonia (BOOP)
• Clinico- Pathological Diagnosis– Clinical– Radiological– Pathological
Brochiolitis Obliterans and Organising Pneumonia (BOOP)
Differential Diagnosis
• Idiopathic (Cryptogenic Organising Pneumonia)
• Infection • Post-transplantation• Collagen vascular disease• Irradiation• Drugs• Other
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.
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.
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
Granulomatosis with polyangiitis (GPA)
Definition
• A multi-organ disease histologically characterized by necrotizing vasculitis involving the small vessels, extensive necrosis & granulomatous inflammation
Granulomatosis with polyangiitis
Clinical manifestationsOrgan involved % at disease onset
% throughout course of disease
Sinusitis 51 85Otitis media 25 44
Pulmonary infiltrates 25 66
Pulmonary nodules 24 58
Hemoptysis 12 30
Glomerulonephritis 18 77
Conjuctivitis, dacrocystitis
5 18
Arthritis, arthralgia 32 67
CNS, peripheral neuropathy
1 15-18
Hoffman GS: Ann Intern Med 1992;116:488
Granulomatosis with polyangiitis
Antineutrophil Cytoplasmic Antibodies (ANCA)
antigenic specificity disease specificty
c-ANCAcytoplasmic proteinase 3
WG (70-93%)
p-ANCAperinuclear
myeloperoxidaseCSS, MPA, PAN,
IBD
Granulomatosis with polyangiitis
Pathology• Necrotizing vasculitis: arterioles,
venules & capillaries• Granulomatous inflammation• Geographical parenchymal
necrosis• Hemorrhagic infarcts• Fibrosis
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%
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
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.
Rheumatoid ArthritisPulmonary manifestations
• Pleural disease pleurisy (20%), effusion (4%), unilateral (80%), R>Lmiddle-aged male, any time during the illnessAssociated with nodules not arthritisFluid: exudate, LDH>1000, low glucose (<50mg% in 80%),low pH, lymphocytic, RF +, low complement
RA-cont.• Interstitial pneumonitis
identical to idiopathic pulmonary fibrosisfullblown in 2%, abnormal PFTs in 41%M>F, patients are RF positiveCough, dyspnea, dry rales, clubbinghypoxemia, restrictive PFTs, reduced DCOVariable response to steroidsPoor prognosis (survival 3-5 years)
May be drug-induced (methotrexate, gold, penicillamine)
RA-cont.• Nodules:
single (1/3) or multiple (2/3), more common in menrelated to disease activity and skin nodulesasymptomatic, may cavitate, rarely hemoptysisperipheral, 0.3-7cm diametermay respond to steroids
• Caplan's syndrome: pulmonary nodules with RA in coal miners (coal miner's pneumoconiosis)
RA-cont.
• Bronchiolitis Obliteransairways obstruction, 60% of smokers, 30% of non-smokersperibronchial inflammatory infiltratemay be drug-induced
• Pulmonary hypertensionpulmonary arteritis
Systemic Lupus ErythematosisPulmonary 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!
Goodpasture's syndrome
• Alveolar hemorrhage• Glomerulonephritis• Anti-glomerular basement
membrane antibody
Goodpasture's syndrome
• Young males• Autoimmune disorder• Clinical features:
Rapidly-progressive glomerulonephritisHematuria, proteinuria, renal failureHemoptysis, dyspnea, coughAnemiaArthralgia
Goodpasture's syndrome• Laboratory features:
X-ray: transient infiltratesUrine:red cells, castsIron-deficiency anemiaRestrictive defect on lung-function tests with increased diffusion capacity.
• Diagnosis:Hemosiderin-laden macrophagesAnti-GBM antibodiesRenal biopsy
• Treatment:Plasmapharesis, steroids, cyclophosphamide.