bronchiolitis obliterans organising pneumonia (boop), granulomatosis with polyangiitis פרופ...
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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.
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