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%. Acute or chronic process with slow progression of airflow obstruction. Fiberoptic endoscopy is most sensitive: flexible rhino laryngoscopy. 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. 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. Atlantoaxial instability C1-C2 instability causing brain stem or spinal cord compression. Prevalence: 25%. Independent of disease duration or patients
age but is most common in patients with severe peripheral joint involvement. Ligamentous laxity induced by inflammation. 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. 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). 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.
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. Pulmonary Renal Syndromes in ICU
Anti GBM Disease Acute Pulmonary hemorrhage, oliguric acute renal failure and anti GBM antibodies. 1 case per 2 million white people. 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
Pathogenesis 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. Anti GBM Disease Hemoptysis: severity is variable.
Alveolar hemorrhage: infectious and non infectious, cigarrete smoking. HLM in alveolar and small airways, interstitial infiltrates, lymphocytic infiltration in alveolar septae and peri broncovascular interstitium. DAD: proteinaceous infiltrates and hyaline membranes.
Anti GBM Disease Crescent formation and GBM destruction. Epithelial crescents in more than 50% of the glomeruli is a poor prognostic factor. 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. Small Vessel Vasculitis Wegener's: c-ANCA or PR3 ANCA (65%) or pANCA or MPO ANCA (20%). Microscopic Polyangiitis and CCS: p-ANCA or MPO ANCA.
Treatment Accurate determination of disease severety. Remission induction phase. Maintenance phase. 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.
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. 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. 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. Role of APL antibodies
Tissue ischemia and necrosis leading to SIRS. SIRS= altered hemostasis, increased coagulation and microvascular fibrin deposition. 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. 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 . Catastrophic Antiphospholipid Syndrome
Valvular vegetations. Coronary artery occlusion with cardiac failure and circulatory collapse. Thrombus formation within the cardiac chambers. Catastrophic Antiphospholipid Syndrome
Renal thrombotic microangiopathy of the glomerular capillaries and small renal arteries. Treatment Anticoagulation and r/o infection. Steroids, plasmapheresis, IVIG.
Plasmaphresis: removal of B2GPI, cytokines and mediators that promote coagulation. SLE SLE Infections is the most common form of
pulmonary involvement. Lupus Pneumonitis. Alveolar Hemorrhage. Acute Reversible Hypoxemia. Shrinking Lung Syndrome. Drug Reaction.
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%
Acute Lupus Pneumonitis Inflammation and tissue injury, no vasculitis: alveolitis, alveolar necrosis, alveolar hemorrhage, edema, interstitial pneumonitis, hyaline membranes, capillary thrombosis, deposition of complement and
immunoglobulins. Acute Lupus Pneumonitis High dose corticosteroids: 1-2 mg/day. Cyclophosphamide, Plasmapheresis. DAH
Mortality 40%-90%. 2% of SLE patients. Young woman with new or establish diagnosis of SLE. Cough, dyspnea, fever, hemoptysis, fall in hematocrit (70%-100%) and pulmonary infiltrates.
Elevated ANA and low complement levels. Active Nephritis. DAH BAL: Increasingly or persistently bloody returns. Mononuclear and polymorphonuclear cell
infiltrates, hyaline membranes, alveolar necrosis, edema, microvascular thrombosis, hemosiderin laden macrophages and capillaritis. DAH High dose corticosteroids ( prednisone 1-3 mg/kg/day
or MTP 1g/day for 3 days followed by prednisone 60mg/day). Adjunctive immunosuppressive ( Cyclophosphamide 500-1000mg/m2/day every 4 weeks) and plasmapheresis ( three to four sessions) in critically ill or non responders. Broad spectrum antibiotics.
In patients on MV, antibiotics were continued until extubation. Drug Reaction Cellular interstitial pneumonia: azathioprine, mycophenolate mofetil. Chronic eosinophilic pneumonia: NSAIDS.
Early onset pneumonitis or upper lobe predominance fibrosis and bilateral pleural thickening: Cyclophosphamide. MTX induced lung injury. Shrinking Lung Syndrome Dyspnea, respiratory muscle dysfunction,
small lung volume, elevated hemi diaphragms, basilar atelectasis. Respiratory muscle weakness, including diaphragmatic dysfunction. Steroids. Acute Reversible Hypoxemia
Active SLE and acute onset of hypoxemia with normal radiologic imaging studies. Endothelial cell and complement-activated neutrophil aggregation within pulmonary capillaries (Pulmonary leuko-aggregation). Elevated A-a gradient, reduced VC and DLCO. Elevated complement degradation products.
Steroids. Neuropsychiatric Manifestations
CVA Transverse Myelitis Seizures Aseptic Meningitis CVA
RR for stroke: 8. 5%-20%. Association with APL antibodies, anti neuronal antibodies, and emboli from cardiac valvular lesions. Transverse Myelitis
Infrequent manifestation. 1%-2%. Early manifestation or within 5 years of diagnosis. Vasculitis or arterial thrombosis associated with APL antibodies. CSF: elevated proteins, pleocytosis, low glucose (<30mg/dl) MRI: Cord edema.
Prednisone, Cyclophosphamide and Plasmapheresis. Seizures Common manifestation. Grand mal seizures are most common. Multifactorial: anti neuronal antibodies, focal
ischemia, infarcts caused by vasculitis or APL antibodies, embolic phenomenon and hemorrhage. Aseptic Meningitis Headache, meningeal signs and CSF pleocytosis (<200-300 cells, lymphocytes).
Respond to corticosteroids. Rule out infectious process, NSAIDS or Azathioprine induced aseptic meningitis. Renal Failure
Drugs: NSAIDs Hypovolemia Sepsis
Previous renal disease. Lupus Nephritis: urinary sediment: proteinuria, casts, RBC. Low complement levels and elevation of anti DNA antibody. SLE/Renal Failure
Before to start aggressive immunosuppression, considerer degree of disease reversibility. Disease reversibility: Renal biopsy?. Scleroderma Limited cutaneous sclerosis (CREST): Raynaud
phenomenon and skin thickening in face and distal extremities. PAH. Diffuse systemic sclerosis: aggressive course, constitutional symptoms, widespread skin thickening, gastrointestinal involvement, pulmonary fibrosis or renal disease.
PAH
Acute Aspiration Alveolar Hemorrhage Renal Crisis PAH Prevalence of PAH is 10-15%. CREST: isolated phenomenon, absence of
pulmonary fibrosis. Diffuse Scleroderma: advanced pulmonary fibrosis. PAH symptoms can predate SSc manifestations. More prevalent in patients with limited cutaneous sclerosis.
PAH Autoimmune process causing damage of vascular endothelium. Transforming Growth Factor Beta and fibroblast proliferation. Infiltration of mononuclear cells, Th2 lymphocytes. High prevalence of anti endothelial cell antibodies (AECA).
IgM AECA has been associated with increase production of endotelin 1. Decreased NO and Prostacyclin. Increased Thromboxane and endotelin 1. Right Ventricular Failure
R/O other causes of RVF: Infectious process
PE Ischemic cardiomyopathy Pericardial tamponade Right Ventricular Failure
Oxygen Diuretics Vasodilators Inotropes
Right Ventricular Failure Inhale NO: less risk for systemic hypotension with significant decrease in PVR, mPAP, RVEDP and increases CO. PGE1: systemic hypotension, mainly in patients with poor vasoreactivity, low CO or
high RAP. Right Ventricular Failure Dobutamine,amrinone,milrinone, dopexamine: inotropic vasodilators. NE rise SBP, does not significantly rise PVR and excellent inotrope.
Pulmonary vasodilators + systemic vasoconstricting inotropes Atrial septostomy? Pulmonary Renal Syndrome Rare complication associated with mortality. Exclusion diagnosis: DAH + ARF.
More than 80% of patients has normal BP vs. hypertensive crises in SSc renal crisis. Associated with MAHA and thrombocytopenia. Segmental necrotizing crescentic glomerulonephritis. Plasma renin activity. Poor response to therapy.
Hypertensive Renal Crisis
Acute malignant hypertension. Diffuse cutaneous disease. High dose corticosteroids. High renin states. Large doses of ACEI. ARB, CCB, prostacyclin, ERA in refractory
cases. Pathogenesis Endothelial cell injury, intimal proliferation with luminal narrowing, decreased renal perfusion, increased renin production, malignant hypertension and RF.
ET-1 Treatment ACEi HD Bibliography
Bibliography Alveolar Hemorrhage in SLE: Presentation and Management. Chest 2000;118;1083-1090. Pulmonary and Thrombotic Manifestations of SLE. Chest 2008;133;271-280. Pulmonary Renal Syndromes in the ICU. Crit
Care Clin.2002 (18):881-895. CAPS. Crit Care Clin.2002(18):805-817.
