1 / 37

Pulmonary vasculitis

Pulmonary vasculitis. Sanitra Sirithangkul M.D. Division of Pulmonary and Critical care Department of Pediatrics Phramongkutklao Hospital. Systemic diseases and the lung. • relatively rare in pediatric practice • CNT diseases : SLE, dermatomyositis • Pulmonary vasculitis

usoa
Download Presentation

Pulmonary vasculitis

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Pulmonary vasculitis Sanitra Sirithangkul M.D. Division of Pulmonary and Critical care Department of Pediatrics Phramongkutklao Hospital

  2. Systemic diseases and the lung • relatively rare in pediatric practice • CNT diseases : SLE, dermatomyositis • Pulmonary vasculitis • Inherited CNT disorders : Ehlers-Danlos • Mucopolysaccharidoses, familial dysautonomia, sarcoidosis •LCH, pulmonary lymphangiomatosis Pediatr Respir Rev 2005;6:181-189

  3. Systemic diseases and the lung • may cause significant respiratory complications • treatment : often requires corticosteroids & immunosuppressants • outcome : variable • when they affect the lung, carry a small but significant mortality Pediatr Respir Rev 2005;6:181-189

  4. Systemic vasculitis •true incidence in children is not known • around 2-5 cases/million/year • pathology of vasculitis : cellular inflammation, vessel destruction, tissue necrosis • clinical features : site, size and type of vessel involved

  5. Classification ของในโรคในกลุ่ม Vasculitis Primary idiopathic vasculitis - Small vessel • Wegener’s granulomatosis • Microscopic polyangitis • Churg-Strauss syndrome • Idiopatic pauci-immune rapidly progressive glomerulonephritis • Isolated pauci-immune pulmonary capillaritis

  6. Primary idiopathic vasculitis - Medium vessel • Polyarteritis nodosa • Kawasaki disease - Large vessel • Giant cell arteritis • Takayasu’ s arteritis Primary immune complex-mediated vasculitis • Goodpasture’s syndrome • Henoch-Schonlein purpura • Behcet’s disease

  7. Classification ของในโรคในกลุ่ม vasculitis (cont.) Secondary vasculitis - Classic autoimmune disease • Systemic lupus erythematosus • Rheumatoid arthritis • Polymyositis / dermatomysitis • Scleroderma • Antiphospholipid antibody syndrome

  8. Secondary vasculitis • Essential cryoglobulinemia • Inflammatory bowel disease • Hypocomplementemic urticarial vasculitis • Drug-induced (e.g., propylthiouracil, diphenylhydantoin) • Paraneoplastic • Infection

  9. Pulmonary vasculitis • one component of systemic vasculitis • pathology : fibrin thrombi, fibrinoid necrosis • the inflammation may lead to - a progressive destruction of pulmonary circulation - granuloma formation - end – organ failure

  10. โรคในกลุ่ม vasculitis ที่ทำให้เกิดพยาธิสภาพที่ปอด • Pulmonary involvement common • - Wegener’s granulomatosis* • - Goodpasture’s syndrome* • - Idiopathic pulmonary hemosiderosis • - Kawasaki disease • * Positive serum anti-neutrophil cytoplasmic antibody (ANCA)

  11. โรคในกลุ่ม vasculitis ที่ทำให้ เกิดพยาธิสภาพที่ปอด (ต่อ) B. Pulmonary involvement uncommon - Henoch-Shonlein purpura - Churg-Strauss vasculitis* - Polyarteritis nodosa* - Takayasu arteritis - Temporal arteritis - Serum sickness - Cryoglobulinemia * Positive serum anti-neutrophil cytoplasmic antibody (ANCA)

  12. Clinical scenarios suggestive of vasculitis 1. Diffuse alveolar hemorrhage (DAH) - hemoptysis - diffuse alveolar infiltration in CXR - a drop in hematocrit

  13. สาเหตุของ Diffuse alveolar hemorrhage With pathologic capillaritis - Primary idiopathic small vessel vasculitis - Primary immune complex-mediated vasculitis - Secondary vasculitis

  14. Without pathologic capillaritis (bland hemorrhage) - Idiopathic pulmonary hemosiderosis - Coagulopathy - Mitral stenosis - Inhalation injury - Goodpasture syndrome - Systemic Lupus erythematosus - Bone marrow transplantation (associated with diffuse alveolar damage) - Drug – associated disease (e.g., chemotherapeutic agents)

  15. Clinical scenarios suggestive of vasculitis 2. Acute glomerulonephritis - rapidly progressive glomerulonephritis (RPGN) - to be considered SLE, post-infectious GN, IgA nephropathy, MPGN, ANCA-associated vasculitis

  16. Clinical scenarios suggestive of vasculitis 3. Pulmonary-renal syndrome - DAH / pulmonary capillaritis + glomerulonephritis 4. Destructive upper airway lesions 5. Chest imaging findings 6. Palpable purpura 7. Mononeuritis multiplex 8. Multisystem disease

  17. Specific testing 1. Antineutrophil cytoplasmic antibodies (ANCA) - circulating autoantibodies against intracellular antigens found in neutrophils - cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA)

  18. แสดงการเปรียบเทียบ c-ANCA และ p-ANCA

  19. แสดงการเปรียบเทียบ c-ANCA และ p-ANCA

  20. Specific testing 2. Radiographic imaging - CT chest : cavity, nodule, diffuse ground glass opacification - CT sinus 3. Bronchoscopy - assess for infection / alveolar hemorrhage / endobronchial lesion

  21. Specific testing 4. Diagnostic biopsy - skin, sinus or upper airway lesions - renal biopsy - lung biopsy - collect tissue in saline for culture - frozen tissue for immunofluorescence - formaline-fixed tissue for H&E

  22. Wegener’s granulomatosis (WG) • the most common of the ANCA-associated vasculitis • triad : upper airway disease, lower respiratory tract disease, glomerulonephritis • abnormal CXR findings : alveolar, interstitial, mixed infiltration nodule/cavity

  23. Wegener’s granulomatosis (WG) • c-ANCA / antiPR3 positive 85-95% of active, systemic WG • poor outcomes : advanced age, severe renal involvement, alveolar hemorrhage, anti PR3 positive

  24. Microscopic polyangiitis (MPA) • long prodromal phase of constitutional symptoms → development of RPGN • pulmonary involvement seen in up to 30% • most common pulmonary involvement : DAH with pulmonary capillaritis

  25. Microscopic polyangiitis (MPA) • p-ANCA +ve 50-70%, anti MPO +ve 35-65%, c-ANCA +ve 10-15% • pathology : focal, segmental necrotizing vasculitis, mixed inflammatory infiltrate without granuloma

  26. Churg-Strauss syndrome (CSS) • to be considered when other eosinophilic lung diseases are in the differential or • difficult-to-control asthmatic patients develop significant cardiac, GI or neurologic disease • triad : asthma, hypereosinophilia, necrotizing vasculitis

  27. Churg-Strauss syndrome (CSS) • pulmonary hemorrhage and glomerulonephritis : less common • p-ANCA /anti MPO +ve 50-75%, c- NCA +ve 10% • mortality & morbidity due to cardiac complications, GI, status asthmaticus & respiratory failure

  28. Therapy •induction of remission : 12 months •maintenance : 12-18 months - cyclophosphamide → azathioprine/ methotrexate - additional agents : mycophenolate mofetil (MMF), leflunomide, cyclosporine - Pneumocystis carinii prophylaxis with trimetroprim-sulfametoxazole

  29. EUVAS grading of disease severity

  30. EUVAS grading of disease severity

  31. Monitoring •to minimize morbidity & mortality of the vasculitides and their therapy •differential diagnosisin pts with clinical deterioration - infection - drug toxicity - disease relapse - a new unrelated problem

  32. Take home message •Pulmonary vasculitis is one component of a variety of systemic vasculitis • Early diagnosis using common clinical scenarios and appropriate investigations

  33. Take home message • Aggressive early treatment to minimize disease related mortality & irreversible damage • Regular monitoring for disease activity and medication toxicity

More Related