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Rheumatology in the ICU

Rheumatology in the ICU. Eduardo Santiago M.D 05/16/2012. Airways Problems in Rheumatologic Disorders. Synovial joints: CA and CT. Laryngeal Involvement in RA. Cricoarytenoid joint arthritis . Long standing RA but also in newly diagnosed patients. Prevalence : 40 %-88%.

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Rheumatology in the ICU

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  1. Rheumatology in the ICU Eduardo Santiago M.D 05/16/2012

  2. Airways Problems in Rheumatologic Disorders

  3. Synovial joints: CA and CT

  4. Laryngeal Involvement in RA • Cricoarytenoid joint arthritis. • Long standing RA but also in newly diagnosed patients. • Prevalence: 40%-88%. • Acute or chronic process with slow progression of airflow obstruction. • Fiberopticendoscopy is most sensitive: flexible rhino laryngoscopy.

  5. Laryngeal Involvement in RA • Intubation can lead to mucosal edema, compromise of airway caliber and stridor and airway obstruction post extubation. • Risk Factors: Laxity of joint capsule and large synovial folds.

  6. Treatment • Severe airway obstruction: Secure airways in OR, Surgeon support, awake fiber optic intubation. • Helium/oxygen mixture improve airflow. • Acute: systemic glucocorticoids, local periarticular steroids can improve CA function. • Chronic: surgery: lateralizing one of the vocal folds, arytenoidectomy vs. tracheostomy.

  7. Atlantoaxial instability • C1-C2 instability causing brain stem or spinal cord compression. • Prevalence: 25%. • Independent of disease duration or patient’s age but is most common in patients with severe peripheral joint involvement. • Ligamentous laxity induced by inflammation.

  8. Atlantoaxial instability • High risk of neurologic injury for cervical manipulation in RA patients. • Avoiding hyperextension and maintaining cervical spine in midline w/o extension. • Awake fiberoptic intubation is recommended.

  9. Wegener Granulomatosis • Major upper airway life threatening complication. • Subglottic stenosis prevalence: 20%. • Acute inflammation or scar formation. • Corticosteroids, immunosuppression, intralesional steroids. • Tracheostomy. • Intratracheal dilation injection ( intralesional long acting corticosteroid injection and mechanical dialtion).

  10. Relapsing Polycondritis • Recurrent episodes of inflammation of cartilaginous and connective tissue structures. • Type II collagenous antibodies. • Respiratory involvement associated with high mortality. • Compromise of glottic, supra or subglottic, trachea and first and second order bronchi.

  11. Relapsing Polycondritis • Encroachment of airway by inflammatory swelling. • Formation of mass of fibrous tissue. • Dissolution of tracheobronchial with subsequent collapse during respiration. • Corticosteroids ( MTP ) and immunosuppression, plasmapheresis. • Surgery: tracheostomy in cases of subglottic involvement. • Endotracheal prostheses and stents.

  12. Pulmonary Renal Syndromes in ICU

  13. Anti GBM Disease • Acute Pulmonary hemorrhage, oliguric acute renal failure and anti GBM antibodies. • 1 case per 2 million white people.

  14. Anti GBM Disease • Type IV collagen. • Alpha chains: alpha1 to 6. • Alpha 3: lung, kidney, seminiferous duct, choroids plexus, optic lens and inner ear. • Autoimmune response to Alpha-3 NC-domain: Anti GBM Syndrome. • Genetic susceptibility: HL DR15, HLA DR 4

  15. Pathogenesis

  16. Anti GBM Disease • Anti GBM antibodies ( anti alpha-3, Type IV antibodies). • Double positive: Anti GBM and ANCA p or ANCA c. • 20%-30% of Anti GBM are double positive, >75% ANCA p positive. • 8-10% ANCA vasculitis are double positive.

  17. Anti GBM Disease • Hemoptysis: severity is variable. • Alveolar hemorrhage: infectious and non infectious, cigarretesmoking. • HLM in alveolar and small airways, interstitial infiltrates, lymphocytic infiltration in alveolar septae and peribroncovascularinterstitium. • DAD: proteinaceous infiltrates and hyaline membranes.

  18. Anti GBM Disease • Crescent formation and GBM destruction. • Epithelial crescents in more than 50% of the glomeruli is a poor prognostic factor.

  19. Therapy • Pulse methylprednisolone, cyclophosphamide and plasmapheresis. • MP: 30mg/kg, on alternate days for 3 doses. Follow by oral prednisone for 12 months. • CP: 2mg/kg, daily dose, should be reduced by 0.5mg/kg every 3 months. • Plasma exchange: 4 liter plasma exchanges daily or on alternate days.

  20. Small Vessel Vasculitis • Wegener's: c-ANCA or PR3 ANCA (65%) or p-ANCA or MPO ANCA (20%). • Microscopic Polyangiitis and CCS: p-ANCA or MPO ANCA.

  21. Treatment • Accurate determination of disease severety. • Remission induction phase. • Maintenance phase.

  22. Treatment • Induction therapy: MP IV 7mg/kg on 3 consecutive days, then prednisone 1mg/kg per day for 1 month, alternate date schedule and the dose is reduced by 10mg/week on the second month. • IV Cyclophosphamide: 0.5mg/kg at monthly intervals ( less side effects than oral) • Oral Cyclophosphamide: 2mg/kg per day.

  23. Treatment • Rituximab (anti CD 20 chimeric monoclonal antibody). • Infliximab. • Plasma exchange or plasmapheresis, twice daily for 7 days in patients with significant pulmonary hemorrhage and renal failure. • IVIG in severe pulmonary hemorrhage.

  24. Catastrophic Antiphospholipid Syndrome • APLS: Thrombosis, thrombocytopenia, recurrent fetal loss and increased APL antibodies. • CAPS: subset characterize with fulminant clinical course with widespread vascular occlusion involving at least three organs in association with ACL or LA. • Thrombocytopenia and MAHA.

  25. Pathology and Pathophysiology • Non inflammatory, thrombotic microangiopathy of small vessels resulting in MOF. • APL: immunoglobulins that bind plasma proteins or phospholipid micro particles. • Activation of platelets, monocytes, tumor cells or endothelial cells leading to pro coagulant state.

  26. Role of APL antibodies • Tissue ischemia and necrosis leading to SIRS. • SIRS= altered hemostasis, increased coagulation and microvascular fibrin deposition.

  27. Catastrophic Antiphospholipid Syndrome • Prior history of APS in 50% to 70%. • Precipitating factors are found in 22%. • Infections, trauma, surgical procedures, tissue biopsies, pregnancy and post fetal demise, malignancy, withdrawal from anticoagulation.

  28. Catastrophic Antiphospholipid Syndrome • Diffuse injury to the capillary endothelial and epithelial cells resulting in ALI and ARDS. • Microvascular thrombi causing endothelial damage, neutrophil influx and cytokines release. • Alveolar hemorrhage .

  29. Catastrophic Antiphospholipid Syndrome • Valvular vegetations. • Coronary artery occlusion with cardiac failure and circulatory collapse. • Thrombus formation within the cardiac chambers.

  30. Catastrophic Antiphospholipid Syndrome • Renal thrombotic microangiopathy of the glomerular capillaries and small renal arteries.

  31. Treatment • Anticoagulation and r/o infection. • Steroids, plasmapheresis, IVIG. • Plasmaphresis: removal of B2GPI, cytokines and mediators that promote coagulation.

  32. SLE

  33. SLE • Infections is the most common form of pulmonary involvement. • Lupus Pneumonitis. • Alveolar Hemorrhage. • Acute Reversible Hypoxemia. • Shrinking Lung Syndrome. • Drug Reaction.

  34. Acute Lupus Pneumonitis • Exclusion diagnosis. Clinical diagnosis. • Dyspnea, cough, fever and hemoptysis. • R/O infection. • Incidence: 0.9-12%. • Initial presentation or in patients who have been already diagnosed with SLE. • Mortality: 50%

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