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Society of General Internal Medicine Society of General Internal Medicine 26th Annual Meeting Vancouver, British Columbia April 30±May 3, 2003 ABSTRACTS AN UNUSUAL CASE OF HEART FAILURE. J. Miller1, S. Agrama1, J. Wald1; 1Temple CLINICAL VIGNETTES University, Philadelphia, PA (Tracking ID #76825) LEARNING OBJECTIVES: Recognize that the presenting features of amyloidosis are protean and depend on the organ systems involved. Recognize that cardiac involvement is common in primary amyloidosis and that symptomatic heart involvement carries a poor prognosis. CASE INFORMATION: 66 year old man without PMH presented 8 months of progressive THE INFILTRATING EOSINOPHIL: A CASE OF CHURG STRAUSS SYNDROME. exertional dyspnea and fatigue. Evaluation revealed volume overload with anasarca and signs of G. Agarwal1, R. Granieri1; 1University of Pittsburgh, Pittsburgh, PA (Tracking ID #73667) biventricular heart failure. LEARNING OBJECTIVES: 1) Recognize the presentation of Churg Strauss Syndrome Laboratory studies revealed renal insufficiency with a creatinine of 1.8 and 2+ proteinuria. (CSS). 2) Diagnose CSS using clinical, laboratory, and histologic parameters. 3) Recognize the serum protein electrophoresis with immunofixation showed a monoclonal IgA kappa treatment of CSS. paraprotein; urine protein electrophoresis was unremarkable. Echocardiogram revealed an CASE INFORMATION: F.T. is a 55-year-old male with adult onset asthma who initially ejection fraction of 20%. Right ventricular function was moderately reduced and right and left presented with progressive asthma exacerbations. A leukotriene inhibitor was added to his ventricular hypertrophy was noted. ECG showed atrial fibrillation with a ventricular response regimen of inhaled beta agonists, and his asthma worsened. He subsequently developed low- of 105 BPM and low voltage. grade fevers, anorexia and a twenty pound weight loss over two months. He also developed Pt. underwent right heart catheterization with endomyocardial biopsy. Tissue stained positive diffuse abdominal pain and hematochezia and was admitted to an outside institution. Colonic with crystal violet and Congo red with characteristic green birefringence under polarization thickening was noted on CT scan, and he was diagnosed with colitis. During this microscopy. This result together with the paraprotein established the diagnosis of AL hospitalization, he suffered a myocardial infarction. A cardiac catheterization revealed clean amyloidosis. coronary arteries. Echocardiogram revealed global hypokinesis, depressed ejection fraction, IMPLICATIONS/DISCUSSION: Amyloidosis is a disease characterized by extracellular and a small pericardial effusion. He was transferred for further workup and management. accumulation of insoluble protein fibrils in various organs and tissues. Classification of Physical exam was notable for thin appearance, mild diffuse abdominal tenderness, mild amyloidosis is based on the nature of the precursor proteins that form the fibril deposits. AL abdominal distension, and sensory loss in his right leg (most notably in the lateral thigh, calf, amyloidosis (primary) is a plasma cell dyscrasia in which clonal plasma cells in the bone marrow and dorsum of his foot). Laboratory work revealed a normochromic normocytic anemia, produce monoclonal immunoglobulin light chains. Clinical manifestations reflect the organ leukocytosis with a marked peripheral eosinophil count, an elevated erythrocyte sedimentation systems involved with kidney and heart involvement being most common. rate (ESR), hypergammaglobulinemia, and an elevated rheumatoid factor level. The Clinically evident cardiac involvement occurs in up to 50% of patients with AL amyloid and antineutrophil cytoplasmic antibody (ANCA) titer was negligible. With a constellation of often leads to significant cardiac dysfunction. Diastolic dysfunction is caused by diffuse adult onset asthma and sinusitis, colitis, possible myocarditis/coronary vasculitis, mild infiltration of the left ventricular myocardium with resultant stiffening and impaired relaxation neuropathy and the above laboratory findings, the patient was diagnosed with CSS. A and usually precedes systolic dysfunction. Systolic dysfunction results from replacement of colonoscopy revealed diffuse colitis and multiple ulcers. Biopsy revealed areas of active colitis functional myocardial tissue with amyloid protein. The conduction system can be affected by with fibrinoid necrosis, fibrin microthrombi in blood vessels and a marked infiltration of amyloid infiltration as well, leading to conduction disturbances, arrhythmias or sudden death. eosinophils. A CT scan of the chest, abdomen, and pelvis revealed pleural effusions, a small ECG findings include low voltage, conduction abnormalities such as AV block, atrial pericardial effusion, and evidence of pneumatosis intestinalis through much of his small bowel. fibrillation and pseudoinfarct patterns. A mesenteric angiogram revealed areas of caliber change, vessel cutoff, and indistinctness of the This case demonstrates a classic presentation of AL amyloidosis with significant cardiac distal small branches of the SMA and IMA, consistent with a vasculitis. The patient was treated involvement. AL amyloidosis has a median survival of one to two years but patients with with high dose steroids and cyclophosphamide and improved significantly. Repeat CT scans symptomatic heart involvement have a median survival of only six months. revealed resolution of the pneumatosis intestinalis. He was discharged with a steroid taper. On follow up, the patient was noted to be doing well, and an echocardiogram showed improvement in his ejection fraction. AN ETHICAL DILEMMA: THE TREATMENT OF COMPLICATIONS OF INTRAVENOUS IMPLICATIONS/DISCUSSION: CSS is a multisystem disorder characterized by asthma, DRUG USE. S. Agresta1, J. Wiese1; 1Tulane University, New Orleans, LA (Tracking peripheral blood eosinophilia, and a systemic vasculitis. CSS can affect multiple organ systems, ID #77219) including the pulmonary, cardiovascular, gastrointestinal, and nervous systems. The incidence, epidemiology, and etiology of CSS remain unclear. CSS does not display a gender LEARNING OBJECTIVES: Recognize the importance of counseling patients who have predominance and can occur at any age. A CSS-like syndrome develops as a rare complication received previous valve replacements that continued intravenous drug use may disqualify them in steroid-dependent asthmatics treated with leukotriene inhibitors. There are three sequential from lifesaving interventions. phases of CSS: 1) a prodromal phase (characterized by atopic disease and asthma), 2) an CASE INFORMATION: A 44 year-old presented with progressive dyspnea, fevers and sub- eosinophilic phase (peripheral eosinophilia and eosinophilic infiltration of organs), and 3) a sternal tightness. He had a endocarditis requiring porcein valve replacement in 1993. He vasculitic phase (a systemic vasculitis of the medium and small vessels). Asthma is the cardinal started using intravenous cocaine and heroin weekly one month prior. He had a fever of 40 C feature, but other features include allergic rhinitis, sinusitis, acute pericarditis, myocarditis, and a holosystolic murmur. Blood cultures grew steptococcus viridans. Transesophageal coronary vasculitis, pulmonary infiltrates, pleural and pericardial effusions, peripheral echocardiography showed aortic valve vegetations on all leaflets and a ring abscess. neuropathies, glomerulonephritis, and eosinophilic gastroenteritis. Although there are no Toxicology screen was positive for cocaine. Ampicillin and gentamicin were initiated. After specific laboratory tests for CSS, peripheral blood eosinophilia is the most characteristic six weeks of therapy, the aortic vegetations and blood cultures persisted. Cardiothoracic finding. Approximately 70% of CSS patients have high ANCA titers. Other nonspecific surgery was consulted, but declined intervention, citing the likely recidivistic drug use and abnormalities include a normochromic, normocytic anemia, an elevated ESR, an elevated IgE futility of care. level, and a positive rheumatoid factor. The diagnosis is suggested clinically and then IMPLICATIONS/DISCUSSION: Surgical intervention in active intravenous drugs users is confirmed by biopsy of affected tissues. Histologic findings include necrotizing vasculitis, an issue of ethical debate. Ninety percent of heroin users will reuse even after successful eosinophilic tissue infiltration, and extravascular granulomas. Most patients respond to rehabilitation. Continued intravenous use represents a hundred-fold increased risk of recurrent corticosteroid therapy. Cyclophosphamide, azathioprine, and high dose intravenous immune endocarditis. The apparent futility of surgical intervention in this setting has prompted surgical globulin have also been used with benefit. Most deaths result from cardiac failure, myocardial services to adopt a ``one strike and youare outpolicy.'' This is almost a certain death sentence infarction, cerebral hemorrhage, renal failure, gastrointestinal bleeding or status asthmaticus. for patients meeting surgical criteria. The ethics committee was involved but could not However, patients that are treated adequately have a survival rate of greater than 70% at establish ethical grounds for challenging surgery's decision. The precedent of withholding five years. organ transplantation from continuing drug users was applied. Physicians should counsel 30 JGIM Volume 18, April (supplement 1) 2003 31 patients who have received previous valve
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