Angiology
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Mucoid Vasculopathy of Unknown Etiology S. Sandhyamani ANGIOLOGY 1991 42: 48 DOI: 10.1177/000331979104200108 The online version of this article can be found at: http://ang.sagepub.com/content/42/1/48
Published by: http://www.sagepublications.com
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Mucoid
Vasculopathy of Unknown Etiology S.
Sandhyamani, M.D. TRIVANDRUM, INDIA
Abstract A new vascular disorder with
generalized deposition of abnormal amounts of acid mucopolysaccharide (AMPS) material in arteries, veins, and vasanervorum has been observed in a large number of autopsies at the author’s institution. It is unlike any of the known vascular diseases and has emerged as a distinct disorder of vascular connective tissue. This has been named "mucoid vasculopathy of unknown etiology." This hitherto unreported entity is described here. Introduction An unusual
distinctive, nonatherosclerotic, and noninflammatory vascular entity has been identified
Occurring in approximately 40% of the autopsies, it is present as a generalized thickening of blood vessels in a large number of young individuals, mostly males, belonging to the lower socio-economic categories irrespective of their primary disease. in autopsy material at
our
center.
Autopsy Findings All blood vessels and their systemic branches throughout their
length are thickened and involved by the arteriopathy (Fig. 1). The arteries feel firm, rubbery, and cartilaginous. There is uniform concentric intimal and medial thickening with consequent diffuse luminal narrowing that becomes accentuated and more obvious after usual fixation procedures. In order to understand the nature of such changes, the sections were studied by means of various staining procedures like hematoxylin and eosin, Alcian Blue, periodic acid Schiff, Oil Red 0, Verhoeff van-Gieson, Alizarin Red, and von Kossa. Histologically, the lesions are characterized by a panarterial deposition of abnormally large amounts of acid mucopolysaccharide (AMPS) or glycosaminoglycan (GAG) material in intima and media of arteries (Fig. 2). Hyperplastic changes are seen in the cellular elements of the arteries. There is no evidence of inflammation or any damage to the fibrous or elastic framework of the arteries. Dystrophic mineralization of internal elastic lamina and of the medial AMPS is a notable feature in some cases. Veins too are thickened and firm and, in some instances, are grossly indistinguishable from arteries (Fig. 3). They show histologic changes similar to those occurring in arteries. Even more impressive is the appearance of vasa-nervorum in the adventitia of some of the affected arteries. This is in the form of lakes of AMPS within nerve bundles (Fig. 4). From the
Department of Pathology, Sree Chitra Tirunal Institue for Medical Sciences and Technology, Trivandrum, India
48
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
49
Fig. narrow
1. Thick-walled branches of renal artery and renal vein have lumina-from a forty years-old male with aortoarteritis.
thick-walled right ovarian vein from a with endomyocardial fibrosis. Pointer shows its site of drainage into the thick-walled opaque inferior vena cava. Ureter (arrow) is cord-like and firm. Aorta (double arrow) has a thick, grey-pink and rubbery wall. Note absence of atherosclerosis in aorta.
Fig. 3. &dquo;Artery-like&dquo; thirty-six-year-old female
......................
2. Superior mesenteric artery from a twenty-six-year-old male with idiopathic aortic regurgitation shows pools of metachromatic acid mucopolysaccharide material in media. Medial cells appear hyperplastic (toluidine blue x 210).
Fig.
Fig. 4. Lake-like deposit of metachromatic acid mucopoly-saccharide material is seen within a nerve bundle in adventitia of aorta (toluidine blue x 210)-from twenty-seven-year-old male with rheumatic heart disease.
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
50 Discussion Vascular lesions described here have not been
reported earlier in the world literature. This entity has
been named &dquo;mucoid vasculopathy of unknown etiology. &dquo; It appears to be peculiar to Kerala, coastal state in the Indian
a southwest
peninsula.
The spectrum of vascular lesions encountered does not resemble any of the conditions known to affect Marfan’s syndrome, arteries, such as, atherosclerosis, aortoarteritis (specific and congenital disorders of AMPS metabolism, 10 Erdheim’s cystic medial necrosis, 11-13 Monckeberg’s scle-
nonspecific),3
rosis with medial calcification,
or even
the natural aging
process.’4 Vascular changes in cases of idiopathic
peripheral limb gangrene in Central Africa resemble the lesions seen in our autopsy material in some aspects. The lesions are also similar to certain degenerative vascular changes observed in experimental nutritional deficiencies.&dquo; Conclusion Detailed
histomorphologic and histochemical studies are currently being carried out at our center to investigate the nature of this entity and to understand the etiopathogenesis of this widespread disorder.
Acknowledgments M.S Valiathan, Director, SCTIMST, Trivandrum for institutional help She wishes to thank the following expert pathologists for reviewing the material and
The author is and
grateful to Dr.
guidance. confirming her observations: Dr. Suman Kinare, Cardiovascular and Thoracic Centre, K.E.M. Hospital, Bombay; Dr. S. Sriramachari, Institute of Pathology (I.C.M.R.), New Delhi; Dr. Carl G. Becker, The Comell Medical Center, New York; Dr. Sheila Haworth, The Hospital for Sick Children, London; and Dr. B. N. Datta, Postgraduate Institute, Chandigarh. The author wishes to thank the Director, Regional Cancer Centre, Trivandrum, for permitting her to take photomicrographs on their microscope. D . Sandhyamani M , Department of Pathology Sree Chitra Tirunal Institute for Medical Sciences and Technology
. S
Trivandrum 695011 , India
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
51
References 1. Haust DM: Atherosclerosis—lesions and sequelae. In: Cardiovascular Pathology, Vol. 1, ed. by Silver M.D. New York: Churchill Livingstone, 1983. 2. Tandon HD, Karmarkar MG, Gupta PK, et al: Morphogenesis of aortic and coronary atherosclerosis in North India—a composite study. Exp Mol Pathol 9:293-308,
1968. 3.
Heggtveit HA: Syphilitic aortitis. autopsy study of 100 cases 1950
A
clinicopathologic
to 1960. Circulation
29:346-355, 1964. 4. Nasu T:
Pathology
of
pulseless
disease.
Angiology
14:225-242, 1963. 5. 6.
Restrepo C. Tajeda C, Correa P: Nonsyphilitic aortitis. Arch Pathol 87:1-12, 1969. Sen PK, Kinare SG, Engineer SD, et al: The middle
aortic syndrome. Br HeartJ 25:610-618, 1963. 7. Isaacson C: An idiopathic aortitis in young Africans. J Path Bact 81:69-79, 1961. 8. Marquis Y. Richardson JB, Ritchie AC, et al: Idiopathic medial aortopathy and arteriopathy. Am J Med 44 :939-
954, 1968. 9. Steiner I, Thomas JD, Hutt MSR: Aortopathies in Ugandan Africans. J Pathol
10. McKusick VA: Heritable disorders of connective tissue. St. Louis: C.V. Mosby Co, 1956. 11. Erdheim J: Medionecrosis aortae idiopathica cystica. Virch Arch Pathol Anat 273:454-479, 1929. 12. Layman TE, Wang Y: Idiopathic cystic medionecrosis and aneurysmal dilation of ascending aorta. Med Clin North Am 52:1145-1156, 1968. 13. Carlson RG, Lillehei CW, Edwards JE: Cystic medial necrosis of the ascending aorta in relation to age and hypertention. Am J Cardiol 25:411-415, 1970. 14. Schlatmann TJH, Becker AE: Histologic changes in the normal aging aorta: Implications for dissecting aortic aneurysm. Am J Cardiol 39:13-20, 1977. 15. Steiner IO, Hutt MSR: Vascularchanges in the idiopathic peripheral gangrene of the tropics. Trop and Geogr Med
24:219-225, 1972. 16. Hutt MSR, Steiner I: Idiopathic peripheral gangrene. In: Cardio- vascular diseases in the tropics, Ed. by Shaper
AG, Hutt MSR, Fejfar Z. London: Br Med Assoc, 1974, pp 369-371. 17. Sriramachari S, Gopalan C: Aortic changes in induced malnutrition. Ind J Med Sci 11:405-409, 1957.
109:295-305, 1973.
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
http://ang.sagepub.com/
Mucoid Vasculopathy of Unknown Etiology S. Sandhyamani ANGIOLOGY 1991 42: 48 DOI: 10.1177/000331979104200108 The online version of this article can be found at: http://ang.sagepub.com/content/42/1/48
Published by: http://www.sagepublications.com
Additional services and information for Angiology can be found at: Email Alerts: http://ang.sagepub.com/cgi/alerts Subscriptions: http://ang.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav Citations: http://ang.sagepub.com/content/42/1/48.refs.html
>> Version of Record - Jan 1, 1991 What is This?
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
Mucoid
Vasculopathy of Unknown Etiology S.
Sandhyamani, M.D. TRIVANDRUM, INDIA
Abstract A new vascular disorder with
generalized deposition of abnormal amounts of acid mucopolysaccharide (AMPS) material in arteries, veins, and vasanervorum has been observed in a large number of autopsies at the author’s institution. It is unlike any of the known vascular diseases and has emerged as a distinct disorder of vascular connective tissue. This has been named "mucoid vasculopathy of unknown etiology." This hitherto unreported entity is described here. Introduction An unusual
distinctive, nonatherosclerotic, and noninflammatory vascular entity has been identified
Occurring in approximately 40% of the autopsies, it is present as a generalized thickening of blood vessels in a large number of young individuals, mostly males, belonging to the lower socio-economic categories irrespective of their primary disease. in autopsy material at
our
center.
Autopsy Findings All blood vessels and their systemic branches throughout their
length are thickened and involved by the arteriopathy (Fig. 1). The arteries feel firm, rubbery, and cartilaginous. There is uniform concentric intimal and medial thickening with consequent diffuse luminal narrowing that becomes accentuated and more obvious after usual fixation procedures. In order to understand the nature of such changes, the sections were studied by means of various staining procedures like hematoxylin and eosin, Alcian Blue, periodic acid Schiff, Oil Red 0, Verhoeff van-Gieson, Alizarin Red, and von Kossa. Histologically, the lesions are characterized by a panarterial deposition of abnormally large amounts of acid mucopolysaccharide (AMPS) or glycosaminoglycan (GAG) material in intima and media of arteries (Fig. 2). Hyperplastic changes are seen in the cellular elements of the arteries. There is no evidence of inflammation or any damage to the fibrous or elastic framework of the arteries. Dystrophic mineralization of internal elastic lamina and of the medial AMPS is a notable feature in some cases. Veins too are thickened and firm and, in some instances, are grossly indistinguishable from arteries (Fig. 3). They show histologic changes similar to those occurring in arteries. Even more impressive is the appearance of vasa-nervorum in the adventitia of some of the affected arteries. This is in the form of lakes of AMPS within nerve bundles (Fig. 4). From the
Department of Pathology, Sree Chitra Tirunal Institue for Medical Sciences and Technology, Trivandrum, India
48
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
49
Fig. narrow
1. Thick-walled branches of renal artery and renal vein have lumina-from a forty years-old male with aortoarteritis.
thick-walled right ovarian vein from a with endomyocardial fibrosis. Pointer shows its site of drainage into the thick-walled opaque inferior vena cava. Ureter (arrow) is cord-like and firm. Aorta (double arrow) has a thick, grey-pink and rubbery wall. Note absence of atherosclerosis in aorta.
Fig. 3. &dquo;Artery-like&dquo; thirty-six-year-old female
......................
2. Superior mesenteric artery from a twenty-six-year-old male with idiopathic aortic regurgitation shows pools of metachromatic acid mucopolysaccharide material in media. Medial cells appear hyperplastic (toluidine blue x 210).
Fig.
Fig. 4. Lake-like deposit of metachromatic acid mucopoly-saccharide material is seen within a nerve bundle in adventitia of aorta (toluidine blue x 210)-from twenty-seven-year-old male with rheumatic heart disease.
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
50 Discussion Vascular lesions described here have not been
reported earlier in the world literature. This entity has
been named &dquo;mucoid vasculopathy of unknown etiology. &dquo; It appears to be peculiar to Kerala, coastal state in the Indian
a southwest
peninsula.
The spectrum of vascular lesions encountered does not resemble any of the conditions known to affect Marfan’s syndrome, arteries, such as, atherosclerosis, aortoarteritis (specific and congenital disorders of AMPS metabolism, 10 Erdheim’s cystic medial necrosis, 11-13 Monckeberg’s scle-
nonspecific),3
rosis with medial calcification,
or even
the natural aging
process.’4 Vascular changes in cases of idiopathic
peripheral limb gangrene in Central Africa resemble the lesions seen in our autopsy material in some aspects. The lesions are also similar to certain degenerative vascular changes observed in experimental nutritional deficiencies.&dquo; Conclusion Detailed
histomorphologic and histochemical studies are currently being carried out at our center to investigate the nature of this entity and to understand the etiopathogenesis of this widespread disorder.
Acknowledgments M.S Valiathan, Director, SCTIMST, Trivandrum for institutional help She wishes to thank the following expert pathologists for reviewing the material and
The author is and
grateful to Dr.
guidance. confirming her observations: Dr. Suman Kinare, Cardiovascular and Thoracic Centre, K.E.M. Hospital, Bombay; Dr. S. Sriramachari, Institute of Pathology (I.C.M.R.), New Delhi; Dr. Carl G. Becker, The Comell Medical Center, New York; Dr. Sheila Haworth, The Hospital for Sick Children, London; and Dr. B. N. Datta, Postgraduate Institute, Chandigarh. The author wishes to thank the Director, Regional Cancer Centre, Trivandrum, for permitting her to take photomicrographs on their microscope. D . Sandhyamani M , Department of Pathology Sree Chitra Tirunal Institute for Medical Sciences and Technology
. S
Trivandrum 695011 , India
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
51
References 1. Haust DM: Atherosclerosis—lesions and sequelae. In: Cardiovascular Pathology, Vol. 1, ed. by Silver M.D. New York: Churchill Livingstone, 1983. 2. Tandon HD, Karmarkar MG, Gupta PK, et al: Morphogenesis of aortic and coronary atherosclerosis in North India—a composite study. Exp Mol Pathol 9:293-308,
1968. 3.
Heggtveit HA: Syphilitic aortitis. autopsy study of 100 cases 1950
A
clinicopathologic
to 1960. Circulation
29:346-355, 1964. 4. Nasu T:
Pathology
of
pulseless
disease.
Angiology
14:225-242, 1963. 5. 6.
Restrepo C. Tajeda C, Correa P: Nonsyphilitic aortitis. Arch Pathol 87:1-12, 1969. Sen PK, Kinare SG, Engineer SD, et al: The middle
aortic syndrome. Br HeartJ 25:610-618, 1963. 7. Isaacson C: An idiopathic aortitis in young Africans. J Path Bact 81:69-79, 1961. 8. Marquis Y. Richardson JB, Ritchie AC, et al: Idiopathic medial aortopathy and arteriopathy. Am J Med 44 :939-
954, 1968. 9. Steiner I, Thomas JD, Hutt MSR: Aortopathies in Ugandan Africans. J Pathol
10. McKusick VA: Heritable disorders of connective tissue. St. Louis: C.V. Mosby Co, 1956. 11. Erdheim J: Medionecrosis aortae idiopathica cystica. Virch Arch Pathol Anat 273:454-479, 1929. 12. Layman TE, Wang Y: Idiopathic cystic medionecrosis and aneurysmal dilation of ascending aorta. Med Clin North Am 52:1145-1156, 1968. 13. Carlson RG, Lillehei CW, Edwards JE: Cystic medial necrosis of the ascending aorta in relation to age and hypertention. Am J Cardiol 25:411-415, 1970. 14. Schlatmann TJH, Becker AE: Histologic changes in the normal aging aorta: Implications for dissecting aortic aneurysm. Am J Cardiol 39:13-20, 1977. 15. Steiner IO, Hutt MSR: Vascularchanges in the idiopathic peripheral gangrene of the tropics. Trop and Geogr Med
24:219-225, 1972. 16. Hutt MSR, Steiner I: Idiopathic peripheral gangrene. In: Cardio- vascular diseases in the tropics, Ed. by Shaper
AG, Hutt MSR, Fejfar Z. London: Br Med Assoc, 1974, pp 369-371. 17. Sriramachari S, Gopalan C: Aortic changes in induced malnutrition. Ind J Med Sci 11:405-409, 1957.
109:295-305, 1973.
Downloaded from ang.sagepub.com at UNIVERSITE LAVAL on June 14, 2014
