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DAH MD - MD seminar Diffuse alveolar hemorrhage hemorrhage...

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Unformatted text preview: MD seminar Diffuse alveolar hemorrhage hemorrhage Definition Medical emergency Medical Pulmonary hemorrhage originating from Pulmonary pulmonary microcirculation (arterioles, capillaries, venules) @ intrapulmonary hemorrhage/pulamonary intrapulmonary alveolar hemorrhage/ pulmonary capillary hemorrhage/ microvascular lung hemorrhage. 1 Etiology With capillaritis Wegener’s granulomatosis Wegener’ Microscopic polyangitis Isolated pulmonary capillaritis Connective tissue disorder Primary antiphospholipid syndrome Mixed cryoglobulinemia Behcets syndrome Henoch scholein purpura Goodpasture’s syndrome Goodpasture’ Pauci-immune glomerulonephritis PauciImmune complex associated glomerulonephritis Drug induced Acute lung allograft rejection Without capillaritis Idiopathic pulmonary hemosiderosis Systemic lupus nephritis Good pasture’s syndrome Diffuse alveolar damage Penicillamine Timellitic anhydride Mitral stenosis Coagulation disorder Pulmonary veno-occlusive disease Pulmonary capillary hemangiomatosis Lymphangioleiomyomatosis Tuberous sclerosis 2 Cardinal symptoms 1. 2. 3. Hemoptysis Shortness of breath Cough recurrent episodes Physical examination Respiratory system: nonspecific Examination of ocular, nasopharynx, cutaneous evidence of vasculitis +/- fever 3 investigation CXR: alveolar infiltrate mostly diffuse + bilateral Pleural effusion rare Blood investigation: Low hemoglobin Raised TLC ANA/ANCA/Antiglomerular basement membrane antibodies ESR Histopathology Alveolar hemorrhage May be due to surgical trauma ( could be differentiated by finding intra alveolar fibrin, hemosiderin in the alveolar walls and hemosiderin-laden macrophages Capillaritis: Fibrin thrombi in capillaries Fibrinoid necrosis of capillary walls Interstitial accumulation of fragmented neutrophils Nuclear dust adjacent to capillaries 4 wegener anemia Renal disease + + cutaneous arthritis + + Micro PAN + + + + SLE + + +/- + Good pasture’s IPH + - - - + - - - IPC + - - - HSP + + + + ANA dsDNA CL RF ANCA ABMA Tissue stain WG +/- - N +- + - granular Mic PAN SLE +- - N +- + - - + + low + - -- granular GP - - N - - + Linear IPH - - N - - - - IPC - - N - +- - - HSP - - N - - - granular 5 Wegener’s granulomatosis Sytemic vasculitis in middle aged adults with necrotizing granulomas in upper and lower respiratory tract cANCA + Focal segmental necrotizing GN DAH with capillaritis(subacute and recurrent) Treated with Corticosteroids and cyclophosphamide Newer agent are IVIG, Cotrimox, Antilymphocyte monoclonal antibodies, tumor necrosis factor inhibitor Microscopic polyangitis Small vessel variant of PAN p ANCA + Focal segmental necrotizing GN DAH with capillaritis is common Treated with corticosteroid + cyclophosphamide /azathioprine Short term mortality is 25% 5 yr survival rate is >60% 6 Good pasture’s syndrome DAH + GN + ABMA in serum / tissue DAH Young smoker Young HLA B7 and HLA DR w2 – severe HLA renal disease and poor prognosis. Treated with Treated corticosteroids/cyclophosphamide/az athioprine/plasmapheresis/ MMF/ anti CD20 Predictors of response - %of Predictors glomerular involvement + renal insufficiency Collagen vascular disease SLE SLE <2% of SLE patients have DAH Has to diff. from lupus pneumonitis Mortality of SLE with DAH is 50% Rheumatoid arthritis Rheumatoid Scleroderma Scleroderma MCTD MCTD polymyositis polymyositis 7 Idiopathic pulmonary hemosiderosis Young children and adults Young Caused: ?linked to Stachybotrys atra Caused: ?immune mediated 20% pediatric patients have LNE and hepatosplenomegaly. Diagnosis of exclusion (needs lung biopsy to prove as bland DAH) Corticosteroid / azathioprine Lung transplant Drugs: penicillamine: DAH+ immune Drugs: complex mediated GN Drugs: with capillaritisDrugs: prophythiouracil, phenytoin, mitomycin. Trimellite anhydride: DAH Trimellite Mitral stenosis: all DAH without Mitral renal/ systemic manifestations needs ECHO to r/o MS. Coagulation disorder: DIC, ITP, Coagulation TTP,acquired vit K defn, APLA syndrome 8 Mixed cryoglobulinemia: Purpura+arthritis+hepatitis+GN A/w Hepatitis B / C infections Leucocyclastic vasculitis. Behcets syndrome: 5-10% have lung manifestations small vessel vasculitis Lymphangioleomyomatosis: premenopausal women proliferation of smooth muscle walls of the pulmonary lymphatics. chylothorax. pneumothorax- 40% hemoptysis:40% focal (DAH is rare) 9 Tuberous sclerosis: 1.mutations in TSC1 and TSC 2 gene 2.triad of mental retardation, epilepsy, derma angiofibroma. 3.lung involvement -1% 4.death due to neurological complication. 10 ...
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