Z Gastroenterol 2009; 47(8): 731-739
DOI: 10.1055/s-0028-1109261
Originalarbeit

© Georg Thieme Verlag KG Stuttgart · New York

Sonographic Signs of Amyloidosis

Sonografische Zeichen der AmyloidoseA. P. Barreiros1 , G. Otto2 , A. Ignee3 , P. Galle1 , C. F. Dietrich3
  • 1Department of Internal Medicine I, Johannes-Gutenberg-University, Mainz, Germany
  • 2Department of Transplantation Surgery, Johannes-Gutenberg-University, Mainz, Germany
  • 3Department of Internal Medicine II, Caritas Krankenhaus, Bad Mergentheim, Germany
Weitere Informationen

Publikationsverlauf

manuscript received: 14.10.2008

manuscript accepted: 2.2.2009

Publikationsdatum:
06. August 2009 (online)

Zusammenfassung

Einleitung: Amyloidosen stellen lebensbedrohliche systemische Erkrankungen dar, die auch junge Patienten betreffen können. Die sonografischen Zeichen sind vielfältig und bisher nicht gut charakterisiert. Patienten: 30 Patienten mit unterschiedlichen Formen einer Amyloidose (19 Patienten ATTR-Amyloidose, 11 Patienten mit anderen generalisierten Amyloidose-Formen) wurden sonografisch evaluiert. Bei allen Patienten wurde die Amyloidose histologisch verifiziert. Ergebnisse: Typische Zeichen des Herzbefalls sind Zeichen einer Polyserositits in Form eines Perikard- und/oder Pleueraergusses sowie die in manchen Fällen sonografisch erkennbaren subendokardialen echoreichen Ablagerungen. Der Darmbefall ist initial kurzstreckig und segmental. Auch hier finden sich typischerweise fokale echoreiche Ablagerungen in der Darmwand. Weitere sonografische Zeichen sind: spontane, variabel echogene subkapsuläre Leber- und Milzeinblutungen sowie vielfältige Nierenveränderungen. Diskussion: Typische sonografisch erkennbare Veränderungen finden sich insbesondere am Herzen, dem unteren Gastrointestinaltrakt, der Leber, der Milz und den Nieren, die in Form eines Pictoral Assays dargestellt werden.

Abstract

Introduction: Amyloidosis is a rare group of diseases with a variety of symptoms that occurs not only in multimorbid elderly but also in young patients. However, sonographic signs of amyloidosis have not been described so far. Patients and Methods: 30 patients with different forms of amyloidosis (19 patients with TTR-amyloidosis (familial amyloid Polyneuropathy [FAP]), 11 patients with other forms of systemic amyloidosis) were sonographically evaluated in a standardised fashion. In all patients amyloidosis was histologically verified. Results: Typical signs of cardiac amyloidosis were myocardial thickness, pericardial effusion, pleural effusion and typical echorich subendocardial depositions. Signs of kidney infiltration were stage dependent and rather unspecific. Spontaneous subcapsular haemorrhages were the typical signs of liver and spleen infiltration. Intestinal affection was characterised by patchy-like inhomogeneous depositions. Rare sites of amyloidal affection could also be verified, e. g. gallbladder and thyroid infiltration. Discussion: Typical sonographic signs of amyloidosis could be found in the heart, bowel wall, liver, spleen, gallbladder and thyroid and were illustrated as pictorial assays.

References

  • 1 WHO-IUIS Nomenclature Sub-Committee . Nomenclature of amyloid and amyloidosis.  Bull World Health Organ. 1993;  71 105-112
  • 2 Dietrich C F, Hirche T O, Schreiber D. et al . Sonographie von Pleura und Lunge.  Ultraschall in Med. 2003;  24 303-311
  • 3 Dietrich C F, Chichakli M, Bargon J. et al . Mediastinal lymph nodes demonstrated by mediastinal sonography: activity marker in patients with cystic fibrosis.  J Clin Ultrasound. 1999;  27 9-14
  • 4 Adibi A, Adibi I, Khosravi P. Do kidney sizes in ultrasonography correlate to glomerular filtration rate in healthy children?.  Australas Radiol. 2007;  51 555-559
  • 5 Dietrich C F, Schall H, Kirchner J. et al . Sonographic detection of focal changes in the liver hilus in patients receiving corticosteroid therapy.  Z Gastroenterol. 1997;  35 1051-1057
  • 6 Dietrich C F, Lee J H, Gottschalk R. et al . Hepatic and portal vein flow pattern in correlation with intrahepatic fat deposition and liver histology in patients with chronic hepatitis C.  Am J Roentgenol. 1998;  171 437-443
  • 7 Gorg C. The forgotten organ: contrast enhanced sonography of the spleen.  Eur J Radiol. 2007;  64 189-201
  • 8 Dietrich C F, Chichakli M, Hirche T O. et al . Sonographic findings of the hepatobiliary-pancreatic system in adult patients with cystic fibrosis.  J Ultrasound Med. 2002;  21 409-416; quiz 417
  • 9 Dietrich C F, Lee J H, Herrmann G. et al . Enlargement of perihepatic lymph nodes in relation to liver histology and viremia in patients with chronic hepatitis C.  Hepatology. 1997;  26 467-472
  • 10 Dietrich C F, Stryjek-Kaminska D, Teuber G. et al . Perihepatic lymph nodes as a marker of antiviral response in patients with chronic hepatitis C infection.  Am J Roentgenol. 2000;  174 699-704
  • 11 Metreweli C, Ward S C. Ultrasound demonstration of lymph nodes in the hepatoduodenal ligament (‘Daisy Chain nodes’) in normal subjects.  Clin Radiol. 1995;  50 99-101
  • 12 Dietrich C F, Zeuzem S. Sonographic detection of perihepatic lymph nodes: technique and clinical value.  Z Gastroenterol. 1999;  37 141-151
  • 13 Hirche T O, Russler J, Schroder O. et al . The value of routinely performed ultrasonography in patients with Crohn disease.  Scand J Gastroenterol. 2002;  37 1178-1183
  • 14 Klein S A, Martin H, Schreiber-Dietrich D. et al . A new approach to evaluating intestinal acute graft-versus-host disease by transabdominal sonography and colour Doppler imaging.  Br J Haematol. 2001;  115 929-934
  • 15 Serin E, Gurel K, Kiyan A. et al . Does diagnostic sonography alter thyroid and parathyroid hormone levels?.  J Clin Ultrasound. 2008;  36 12-15
  • 16 Cook G C. Recurrent hereditary polyserositis or familial Mediterranean fever: An overview.  Ann Saudi Med. 1991;  11 576-584
  • 17 Rocken C, Sletten K. Amyloid in surgical pathology.  Virchows Arch. 2003;  443 3-16
  • 18 Subramanyam B R. Renal amyloidosis in juvenile rheumatoid arthritis: sonographic features.  Am J Roentgenol. 1981;  136 411-412
  • 19 Beyer D, Krug B, Stelzner M. Gastrointestinal amyloidosis as a differential diagnostic problem.  Röntgenstr Fortschr. 1986;  145 551-555
  • 20 Narumi H, Funabashi N, Takano H. et al . Remarkable thickening of right atrial wall in subjects with cardiac amyloidosis complicated with sick sinus syndrome.  Int J Cardiol. 2007;  119 222-224
  • 21 Kyle R A, Greipp P R, Banks P M. The diverse picture of gamma heavy-chain disease. Report of seven cases and review of literature.  Mayo Clin Proc. 1981;  56 439-451
  • 22 Cueto-Garcia L, Reeder G S, Kyle R A. et al . Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival.  J Am Coll Cardiol. 1985;  6 737-743
  • 23 Selvanayagam J B, Hawkins P N, Paul B. et al . Evaluation and management of the cardiac amyloidosis.  J Am Coll Cardiol. 2007;  50 2101-2110
  • 24 Ulucam M, Yildirir A, Muderrisoglu H. et al . Doppler tissue imaging of the heart in secondary amyloidosis.  Adv Ther. 2005;  22 433-442
  • 25 Falk R H, Plehn J F, Deering T. et al . Sensitivity and specificity of the echocardiographic features of cardiac amyloidosis.  Am J Cardiol. 1987;  59 418-422
  • 26 Siqueira-Filho A G, Cunha C L, Tajik A J. et al . M-mode and two-dimensional echocardiographic features in cardiac amyloidosis.  Circulation. 1981;  63 188-196
  • 27 Comenzo R L, Vosburgh E, Falk R H. et al . Dose-intensive melphalan with blood stem-cell support for the treatment of AL (amyloid light-chain) amyloidosis: survival and responses in 25 patients.  Blood. 1998;  91 3662-3670
  • 28 Mikami Y, Funabashi N, Kijima T. et al . Focal fibrosis in the left ventricle of subjects with cardiac amyloidosis evaluated by multislice computed tomography.  Int J Cardiol. 2007;  122 72-75
  • 29 Krombach G A, Hahn C, Tomars M. et al . Cardiac amyloidosis: MR imaging findings and T 1 quantification, comparison with control subjects.  J Magn Reson Imaging. 2007;  25 1283-1287
  • 30 Sueyoshi E, Sakamoto I, Okimoto T. et al . Cardiac amyloidosis: typical imaging findings and diffuse myocardial damage demonstrated by delayed contrast-enhanced MRI.  Cardiovasc Intervent Radiol. 2006;  29 710-712
  • 31 Maceira A M, Joshi J, Prasad S K. et al . Cardiovascular magnetic resonance in cardiac amyloidosis.  Circulation. 2005;  111 186-193
  • 32 Perugini E, Rapezzi C, Piva T. et al . Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance.  Heart. 2006;  92 343-349
  • 33 Ekelund L. Radiologic findings in renal amyloidosis.  Am J Roentgenol. 1977;  129 851-853
  • 34 Moon W K, Kim S H, Im J G. et al . Castleman disease with renal amyloidosis: imaging findings and clinical significance.  Abdom Imaging. 1995;  20 376-378
  • 35 Apter S, Zemer D, Terhakopian A. et al . Abdominal CT findings in nephropathic amyloidosis of familial Mediterranean fever.  Amyloid. 2001;  8 58-64
  • 36 Kim S H, Han J K, Lee K H. et al . Abdominal amyloidosis: spectrum of radiological findings.  Clin Radiol. 2003;  58 610-620
  • 37 Buck F S, Koss M N. Hepatic amyloidosis: morphologic differences between systemic AL and AA types.  Hum Pathol. 1991;  22 904-907
  • 38 Bujanda L, Beguiristain A, Alberdi F. et al . Spontaneous rupture of the liver in amyloidosis.  Am J Gastroenterol. 1997;  92 1385-1386
  • 39 Gastineau D A, Gertz M A, Rosen C B. et al . Computed tomography for diagnosis of hepatic rupture in primary systemic amyloidosis.  Am J Hematol. 1991;  37 194-196
  • 40 Okazaki K, Moriyasu F, Shiomura T. et al . Spontaneous rupture of the spleen and liver in amyloidosis – a case report and review of the literature.  Gastroenterol Jpn. 1986;  21 518-524
  • 41 Balakrishnan C, Sule A, Mittal G. et al . Treatment of AA amyloidosis in rheumatoid arthritis.  J Assoc Physicians India. 2002;  50 907-909
  • 42 Choufani E B, Sanchorawala V, Ernst T. et al . Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy.  Blood. 2001;  97 1885-1887
  • 43 Casillas V J, Amendola M A, Gascue A. et al . Imaging of nontraumatic hemorrhagic hepatic lesions.  Radiographics. 2000;  20 367-378
  • 44 Mukhopadhya A, Raghuram L, Justus A. et al . Transcatheter hepatic artery embolization for spontaneous rupture of amyloid liver.  Indian J Gastroenterol. 2004;  23 26-27
  • 45 Turner C A, Tung K. CT appearances of amyloid lymphadenopathy in a patient with non-Hodgkin’s lymphoma.  Br J Radiol. 2007;  80 e250-e252
  • 46 Kwon A H, Tsuji K, Yamada H. et al . Amyloidosis of the gallbladder mimicking gallbladder cancer.  J Gastroenterol. 2007;  42 261-264
  • 47 Casassus-Builhe D, Chauveau E, Bechade D. et al . Systemic amyloidosis: localization in the gallbladder.  Presse Med. 2000;  29 306
  • 48 Remy A J, Perney P, Bourat L. et al . Amyloidosis of the gallbladder. An unusual localization.  Gastroenterol Clin Biol. 1995;  19 215-217
  • 49 Urban B A, Fishman E K, Goldman S M. et al . CT evaluation of amyloidosis: spectrum of disease.  Radiographics. 1993;  13 1295-1308
  • 50 Monzawa S, Tsukamoto T, Omata K. et al . A case with primary amyloidosis of the liver and spleen: radiologic findings.  Eur J Radiol. 2002;  41 237-241
  • 51 Suzuki S, Takizawa K, Nakajima Y. et al . CT findings in hepatic and splenic amyloidosis.  J Comput Assist Tomogr. 1986;  10 332-334
  • 52 Benson L, Hemmingsson A, Ericsson A. et al . Magnetic resonance imaging in primary amyloidosis.  Acta Radiol. 1987;  28 13-15
  • 53 Moller J M, Santoni-Rugiu E, Chabanova E. et al . Magnetic resonance imaging with liver-specific contrast agent in primary amyloidosis and intrahepatic cholestasis.  Acta Radiol. 2007;  48 145-149
  • 54 Bandyopadhyay S K, Bandyopadhyay R, Dutta A. Primary amyloidosis presenting as intrahepatic cholestasis.  Indian J Pathol Microbiol. 2006;  49 557-559
  • 55 Mainenti P P, Camera L, Nicotra S. et al . Splenic hypoperfusion as a sign of systemic amyloidosis.  Abdom Imaging. 2005;  30 768-772
  • 56 Gilat T, Revach M, Sohar E. Deposition of amyloid in the gastrointestinal tract.  Gut. 1969;  10 98-104
  • 57 Bedioui H, Chebbi F, Ayadi S. et al . Gastric amyloidosis mimicking malignancy. A case report.  Ann Chir. 2006;  131 455-458
  • 58 Akl M N, Kho R M, McCullough A E. et al . Mesenteric and omental amyloidosis mimicking intraperitoneal carcinomatosis.  Surgery. 2008;  144 473-475
  • 59 Dietrich C F, Brunner V, Lembcke B. Intestinal ultrasound in rare small and large intestinal diseases.  Z Gastroenterol. 1998;  36 955-970
  • 60 Watanabe T, Kato K, Sugitani M. et al . A case of solitary amyloidosis localized within the transverse colon presenting as a submucosal tumor.  Gastrointest Endosc. 1999;  49 644-647
  • 61 Kala Z, Valek V, Kysela P. Amyloidosis of the small intestine.  Eur J Radiol. 2007;  63 105-109
  • 62 Araoz P A, Batts K P, MacCarty R L. Amyloidosis of the alimentary canal: radiologic-pathologic correlation of CT findings.  Abdom Imaging. 2000;  25 38-44
  • 63 Yoshimatsu S, Ando Y, Terazaki H. et al . Endoscopic and pathological manifestations of the gastrointestinal tract in familial amyloidotic polyneuropathy type I (Met30).  J Intern Med. 1998;  243 65-72
  • 64 Komuro O, Murakami S, Kobayashi T. et al . An autopsy case of AL amyloidosis with intestinal pseudo-obstruction.  Nippon Shokakibyo Gakkai Zasshi. 2005;  102 699-705
  • 65 Andrews C N, Amar J N, Hayes M M. et al . Systemic amyloidosis: a rare presentation of mesenteric angina.  Can J Gastroenterol. 2002;  16 683-685
  • 66 Siddiqui M A, Gertz M, Dean D. Amyloid goiter as a manifestation of primary systemic amyloidosis.  Thyroid. 2007;  17 77-80
  • 67 Fontan F J, Cordido F, Mosquera J. et al . Amyloid goitre: CT and MR findings.  Clin Radiol. 1995;  50 409-411
  • 68 Miyake H, Maeda H, Isomoto I. et al . Computed tomography in amyloid goiter.  J Comput Assist Tomogr. 1988;  12 621-622
  • 69 Laly F, Duveau J P, Behar F. et al . Cerebral amyloid angiopathy.  Psychol Neuropsychiatr Vieil. 2007;  5 35-45
  • 70 Chao C P, Kotsenas A L, Broderick D F. Cerebral amyloid angiopathy: CT and MR imaging findings.  Radiographics. 2006;  26 1517-1531

Prof. Dr. Christoph F. Dietrich

Department of Internal Medicine II, Caritas Krankenhaus Bad Mergentheim

Uhlandstr. 7

97980 Bad Mergentheim

Germany

eMail: christoph.dietrich@ckbm.de