Pulmonary hypertension leading to right heart failure in a patient with iga gammopathy
1nternal Medicine, St Elizabeth’s Hospital, Belmont Avenue, Youngstown
DOI: 10.22514/SV21.042007.7 Vol.2,Issue 1,April 2007 pp.29-32
Published: 20 April 2007
Amyloidosis is a rare disorder characterized by the deposition of amorphous, extracellular, insoluble fibrillar protein in various tissues of the body. Pulmonary hypertension usually occurs in the last stages of the disease with co-existing left ventricular failure. Amyloidosis causing pulmonary hypertension in a patient with no evidence of left ventricular failure is rarely mentioned in literature. Here, we present a patient with IgA gammopathy presenting with pulmonary hypertension leading to progressive right heart failure and death.
amyloidosis, pulmonary hypertension, IgA gammopathy
YASHVIR S. SANGWAN,BHAGWAN DASS. Pulmonary hypertension leading to right heart failure in a patient with iga gammopathy. Signa Vitae. 2007. 2(1);29-32.
01. Gertz MA, Kyle RA. Primary systemic amyloidosis – a diagnostic primer. Mayo Clin Proc 1989;64:1505-19.
02. Kyle RA, Greipp PR. Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc 1983;58:665-83.
03. Hawkins PN. The diagnosis, natural history and treatment of amyloidosis. J R Coll Physicians Lond 1997;31:552-60.
04. Pascali E. Diagnosis and treatment of primary amyloidosis. Crit Rev Oncol Hematol 1995;19:149-81.
05. Kyle RA, Gertz MA. Primary systemic amyloidosis clinical and laboratory features in 474 cases. Semin Hematol 1995;32:45-59.
06. Gertz MA, Kyle RA, Greipp PR. Response rates and survival in primary systemic amyloidosis. Blood 1991;77:257-62.
07. Gertz MA, Greipp PR, Kyle RA. Classification of amyloidosis by the detection of clonal excess of plasma cells in the bone marrow. J Lab Clin Med 1991;118:33-9.
08. Wu SS, Brady K, Anderson JJ, Vezina R, Skinner M, Neiman RS, et al. The predictive value of bone marrow morphologic characteristics and immunostaining in primary (AL) amyloidosis. Am J Clin Pathol 1991;96:95-9.
09. Westermark P. Diagnosing amyloidosis. Scand J Rheumatol. 1995;24:327-9.
10. Gertz MA, Grogan M, Kyle RA, Tajik AJ. Endomyocardial biopsy-proven light chain amyloidosis (AL) without echocardiographic features of infiltrative cardiomyopathy. Am J Cardiol 1997;80:93-5.
11. Gillmore JD, Hawkins PN, Pepys MB. Amyloidosis: a review of recent diagnostic and therapeutic developments. Br J Haematol 1997;99:245-56.
12. Schena FP, Pannarale G, Carbonara MC. Clinical and therapeutic aspects of renal amyloidosis. Nephrol Dial Transplant 1996;1l:63-8.
13. Peters RA, Koukoulis G, Gimson A, Portmann B, Westaby D, Williams R. Primary amyloidosis and severe intrahepatic cholecystatic jaun-dice. Gut 1994;35:1322-5.
14. Rajkumar SV, Gertz MA, Kyle RA. Prognosis of patients with primary systemic amyloidosis who present with dominant neuropathy. Am J Med 1998;104:232-7.
15. Gertz MA, Kyle RA. Prognostic value of urinary protein in primary systemic amyloidosis. Am J Clin Pathol 1990;94:313-7.
16. Gertz MA, Kyle RA, O’Fallon WM. Dialysis support of patients with primary systemic amyloidosis. A study of 211 patients. Arch Intern Med 1992;152:2245-50.
17. Siqueira-Filho AG, Cunha CL, Tajik AJ, Seward JB, Schattenberg TT, Giuliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1981;63:188-96.
18. Klein AL, Hatle LK, Burstow DJ, Seward JB, Kyle RA, Bailey KR, et al. Doppler characterization of left ventricular diastolic function in cardiac amyloidosis. J Am Coll Cardiol 1989;13:1017-26.
19. Falk RH, Plehn JF, Deering T, Schick EC Jr, Boinay P, Rubinow A, et al. Sensitivity and specificity of the echocardiographic features of cardiac amyloidosis. Am J Cardiol 1987;59:418-22.
20. Hamer JP, Janssen S, Van Rijswijk MH, Lie KI. Amyloid cardiomyopathy in systemic non-hereditary amyloidosis. Clinical, echocardio-graphic and electrocardiographic findings in 30 patients with AA and 24 patients with AL amyloidosis. Eur Heart J 1992;13:623-7.
21. Hancock EW. Low voltage, Q waves, and congestive heart failure. Hosp Pract (Minneap) 1997;32:21-2.
22. Gertz MA, Kyle RA. Hepatic amyloidosis: clinical appraisal in 77 patients. Hepatology 1997;25:118-21.
23. Bujanda L, Beguiristain A, Alberdi F, Cosme A, Ruiz de la Hermosa J, Gutierrez-Stampa, et al. Spontaneous rupture of liver in amyloidosis. Am J Gastroenterol 1997;92:1385-6.
24. Gastineau DA, Gertz MA, Rosen CB, Kyle RA. Computed tomography for diagnosis of hepatic rupture in primary systemic amyloidosis. Am J Hematol 1991;37:194-6.
25. Richey TK, Bennion SD. Etiologies of the sicca syndrome: primary systemic amyloidosis and others. Int J Dermatol 1996;35:553-7.
26. Schlesinger I. Multiple myeloma and AL amyloidosis mimicking Sjogren’s syndrome. South Med J. 1993;86:568-9.
27. Menke DM, Kyle RA, Fleming CR, Wolfe JT 3rd, Kurtin PJ, Oldenburg WA. Symptomatic gastric amyloidosis in patients with primary sys-temic amyloidosis. Mayo Clin Proc 1993;68:763-7.
28. Fraser AG, Arthur JF, Hamilton I. Intestinal pseudoobstruction secondary to amyloidosis responsive to cisapride. Dig Dis Sci 1991;36:532-5.
29. Trinh TD, Jones B, Fishman EK. Amyloidosis of the colon presenting as ischemic colitis: a case report and review of the literature. Gas-trointest Radiol 1991;16:133-6.
30. Lee JG, Wilson JA, Gottfried MR. Gastrointestinal manifestations of amyloidosis. South Med J 1994;87:243-7.
31. Gertz MA, Kyle RA. Myopathy in primary systemic amyloidosis. J Neurol Neurosurg Psychiatry 1996;60:655-60.
32. Katz GA, Peter JB, Pearson CM, Adams WS. The shoulder pad sign – a diagnostic feature of amyloid arthropathy. N Engl J Med 1973;288:354-5.
33. Rao JK, Allen NB. Primary systemic amyloidosis masquerading as giant cell arteritis. Case report and review of the literature. Arthritis Rheum 1993;36:422-5.
34. Rodon P, Friocourt P, Blanchet S, Levallois D. Temporal artery involvement revealing AL amyloidosis and IgD monoclonal gammopathy. J Rheumatol 1996;23:189-90.
35. Salvarani C, Gabriel SE, Gertz MA, Bjornsson J, Li CY, Hunder GG. Primary systemic amyloidosis presenting as giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum 1994;37:1621-6.
36. Rubinow A, Celli BR, Cohen AS, Rigden BG, Brody JS. Localized amyloidosis of the lower respiratory tract. Am Rev Respir Dis 1978;118:603-11.
37. Smith RR, Hutchins GM, Moore GW, Humphrey RL. Type and distribution of pulmonary parenchymal and vascular amyloid. Am J Med 1979;66:96-104.
38. Gastineau DA, Gertz MA, Daniels TM, Kyle RA, Bowie EJ. Inhibitor of thrombin time in systemic amyloidosis. Blood 1991;77:2637-40.
39. Mizutani AR, Ward CF. Amyloidosis associated bleeding diathesis in the surgical patient. Can J Anaesth 1990;37:910-2.
40. Shiue ST, McNally DP. Pulmonary hypertension from prominent vascular involvement in diffuse amyloidosis. Arch Intern Med 1988;148:687-9.
41. Gertz MA, Kyle RA. Amyloidosis: prognosis and treatment. Semin Arthritis Rheum 1994;24:124-38.
42. Kyle RA, Greipp PR, O’Fallon WM. Primary systemic amyloidosis: multivariate analysis for prognostic factors in 168 cases. Blood 1986;68:220-4.
43. Chamarthi B, Dubrey SW, Cha K, Skinner M, Falk RH. Features and prognosis of exertional syncope in light-chain associated AL cardiac amyloidosis. Am J Cardiol 1997;80:1242-5.
44. Gertz MA, Lacy MQ, Dispenzeiri A: Amyloidosis. Hematol Oncol Clin North Am 1999;13:1211-33.
45. Kyle RA, Gertz MA, Greipp PR, Witzig TE, Lust JA, Lacy MQ, et al. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med 1997;336:1202-7.
46. Dubrey S, Simms RW, Skinner M, Falk RH. Recurrence of primary (AL) amyloidosis in a transplanted heart with four-year survival. Am J Cardiol 1995;76:739-41.
47. Pelosi F Jr, Capehart J, Roberts WC. Effectiveness of cardiac transplantation for primary (AL) cardiac amyloidosis. Am J Cardiol 1997;79:532-5.
48. van Buren M, Hene RJ, Verdonck LF, Verzijlbergen FJ, Lokhorst HM. Clinical remission after syngeneic bone marrow transplantation in a patient with AL amyloidosis. Ann Intern Med 1995;122:508-10.
49. Moreau P, Leblond V, Bourquelot P, Facon T, Huynh A, Caillot D, et al. Prognostic factors for survival and response after high-dose therapy and autologous stem cell transplantation in systemic AL amyloidosis: a report on 21 patients. Br J Haematol 1998;101:766-9.
Science Citation Index Expanded (SciSearch) The Science Citation Index (SCI) is a citation index originally produced by the Institute for Scientific Information (ISI) and created by Eugene Garfield. It (Science Citation Index Expanded) covers more than 8,500 notable and significant journals, across 150 disciplines in science and technology, from 1900 to the present.
Journal Citation Reports/Science Edition Journal Citation Reports/Science Edition aims to evaluate a journal’s value from multiple perspectives including the journal impact factor, descriptive data about a journal’s open access content as well as contributing authors, and provide readers a transparent and publisher-neutral data & statistics information about the journal.
Chemical Abstracts Service Source Index The CAS Source Index (CASSI) Search Tool is an online resource that can quickly identify or confirm journal titles and abbreviations for publications indexed by CAS since 1907, including serial and non-serial scientific and technical publications.
IndexCopernicus The Index Copernicus International (ICI) Journals database’s is an international indexation database of scientific journals. It covered international scientific journals which divided into general information, contents of individual issues, detailed bibliography (references) sections for every publication, as well as full texts of publications in the form of attached files (optional). For now, there are more than 58,000 scientific journals registered at ICI.
Geneva Foundation for Medical Education and Research The Geneva Foundation for Medical Education and Research (GFMER) is a non-profit organization established in 2002 and it works in close collaboration with the World Health Organization (WHO). The overall objectives of the Foundation are to promote and develop health education and research programs.
Scopus Scopus is Elsevier's abstract and citation database launched in 2004. Scopus covers nearly 36,377 titles (22,794 active titles and 13,583 Inactive titles) from approximately 11,678 publishers, of which 34,346 are peer-reviewed journals in top-level subject fields: life sciences, social sciences, physical sciences and health sciences.
Embase Embase (often styled EMBASE for Excerpta Medica dataBASE), produced by Elsevier, is a biomedical and pharmacological database of published literature designed to support information managers and pharmacovigilance in complying with the regulatory requirements of a licensed drug.