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Monckeberg's arteriosclerosis

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Mönckeberg's sclerosis
Other namesMedial calcific sclerosis or Mönckeberg medial sclerosis
Microphotography of arterial wall with calcified (violet colour) atherosclerotic plaque (haematoxylin & eosin stain)
SpecialtyCardiology Edit this on Wikidata
A. Pelvic and lower extremity radiograph shows extensive calcification of the femoral arteries. B. Translumbar aortography shows near-total obstruction of the femoral arteries.

Mönckeberg's arteriosclerosis, or Mönckeberg's sclerosis, is a non-inflammatory form of arteriosclerosis or vessel hardening, where calcium deposits are found in the muscular middle layer of the walls of arteries (the tunica media).[1] It is an example of dystrophic calcification. This condition occurs as an age-related degenerative process. However, it can occur in pseudoxanthoma elasticum and idiopathic arterial calcification of infancy as a pathological condition, as well. Its clinical significance and cause are not well understood and its relationship to atherosclerosis and other forms of vascular calcification are the subject of disagreement.[2] Mönckeberg's arteriosclerosis is named after Johann Georg Mönckeberg,[3][4] who first described it in 1903.

The severity of calcium deposits formed by Mönckeberg's arteriosclerosis can be categorized into stages based on the histological appearance. Understanding these stages can help to understand disease progression and how the disease is caused. Stage 1 involves the formation of calcium deposits both inside and outside the vascular smooth muscle cells which compose the tunica media. Calcification outside of the vascular smooth muscle cells are commonly associated with damage to elastic fibers in the extra-cellular matrix. These calcium deposits also develop on the internal elastic lamina. Stage 2 and Stage 3 involve the formation of calcified sheaths spanning an increased diameter through the tunica media. Stage 4 then involves the formation of bony tissue from these calcifications through the process of osseous metaplasia.[5][6]

Signs and symptoms

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Typically, Mönckeberg's arteriosclerosis is not associated with symptoms unless complicated by atherosclerosis, calciphylaxis, or accompanied by some other disease.[7] However, the presence of Mönckeberg's arteriosclerosis is associated with poorer prognosis.[8] This is probably due to vascular calcification causing increased arterial stiffness, increased pulse pressure and resulting in exaggerated damage to the heart and kidneys.[9] The clinical symptoms of Mönckeberg's arteriosclerosis are similar to giant cell arteritis in which the two can be mistakenly interchanged. A temporal artery biopsy (TAB) can be performed to differentiate between the two disease states.[10]

Mönckeberg's arteriosclerosis may also be a risk factor for the development of cranial infarctions. Intercranial Mönckeberg's arteriosclerosis has been commonly found in people with malignant tumors who died of cerebral infarctions. In cranial autopsies, areas of Mönckeberg's arteriosclerosis were located near the sites of infarction.[11]

Cause

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Minor degrees of calcification of the cardiovascular system are common in elderly people,[12] and the prevalence of vascular calcification is increased by some diseases (see Epidemiology section). Vascular calcification results from the deposition of calcium phosphate crystals (hydroxyapatite) as a consequence of disordered calcium phosphate regulation in the blood vessel. Hydroxyapatite is secreted in vesicles that bleb out from vascular smooth muscle cells or pericytes in the arterial wall.[2] The mechanism of vascular calcification is not fully understood, but probably involves a phenotypic change in the vascular smooth muscle cells in the wall with activation of bone-forming programs. Numerous regulators of calcification such as osteopontin, osteoprotegerin, matrix gla protein and fetuin-A, receptor activator of NF-kappa-B, receptor activator of NF-kappa-B ligand and tumor necrosis factor (TNF)-related apoptosis-inducing ligand protein have been implicated in this process.[13][14]

It is unclear whether Mönckeberg's arteriosclerosis is a distinct entity or forms part of a spectrum of vascular calcification that includes atherosclerosis and calcification in the inner layer of the artery wall (tunica intima), calcification of the internal elastic lamina, calcification of cardiac valves and widespread soft tissue calcification.[15] The existence of Mönckeberg's arteriosclerosis has been disputed and it has been proposed that it is a part of a continuum of atherosclerotic disease:[13] the majority of atherosclerotic plaques contain some calcium deposits[16][17] and calcification of the internal elastic lamina is common in pathological specimens labelled as Mönckeberg's arteriosclerosis.[2] However studies in animals suggest that a predominantly medial pattern of vascular calcification reflects different underlying mechanisms of disease,[12] and despite involvement of the internal elastic lamina, evidence of inflammation is rare in Mönckeberg's arteriosclerosis.[2]

Diagnosis

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Diagnosis of this rare disease is often misdiagnosed or delayed, leading to results such as amputation and death. Due to this, it is important to utilize comprehensive medical testing, examination, and diagnostic tests.[18] Often Mönckeberg's arteriosclerosis is discovered as an incidental finding in an X-ray radiograph, on mammograms, in autopsy, or in association with investigation of some other disease, such as diabetes mellitus or chronic kidney disease. The diagnosis is usually confirmed by a radiography result or an ultrasonography.[19] Typically calcification is observed in the arteries of the upper and lower limb although it has been seen in numerous other medium size arteries.[7] In the radial or ulnar arteries it can cause "pipestem" arteries, which present as a bounding pulse at the end of the calcific zone. It may also result in "pulselessness." Epidemiological studies have used the ratio of ankle to brachial blood pressure (ankle brachial pressure index, ABPI or ABI) as an indicator of arterial calcification with ABPI >1.3 to >1.5 being used as a diagnostic criterion depending on the study.[20][21]

Management

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Currently, there are no guideline therapies established to treat Mönckeberg's arteriosclerosis. There have been more studies as of recently to learn more about the disease and potential pharmacological managements. Recent studies that are showing potential emerging therapies that can help treat arteriosclerosis. A therapy using vasostatin-1, which is a chromogranin A derived peptide, has shown potential in helping treat Mönckeberg's arteriosclerosis.[22]

There are some recommendations for the clinical treatment for people who have complications related to Mönckeberg's arteriosclerosis. Specifically for people with underlying phosphate disorders, the use of phosphate binders, low-dose vitamin D, calcimimetics, magnesium, bisphosphonates, sodium thiosulfate, and aldosterone antagonists have been proposed. Lowering calcium and phosphate levels in people with calciphylaxis, along with increasing hemodialysis and treating potential ischemic necrosis is also recommended.[23]

There are still further discussions and trials needed to help define treatment goals for Mönckeberg's arteriosclerosis.

Clinical Studies and Case Report

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75 Year Old Male

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A 75-year-old South Asian male presents with complaints of an unilateral headache and overall head and facial pain, with specific pain centered on the left side of their neck. The individual had first noticed the pain about two weeks before their visit to the clinic. The pain was described to be ongoing and continuous throughout the day and rated 6-7 on the numerical visual analog scale (VAS). They reported no relieving factors and multiple different associated factors including scalp tenderness and non-specific shoulder pain. The individual had tried multiple pain medications medications ranging from over-the-counter medications (OTC) to prescription medications including pregabalin and indomethacin but had reported to receive no pain relief from any of the medications they had taken. An orofacial pain evaluation was performed which resulted in no abnormalities found in the patient. The individual had their pain, which was produced by Mönckeberg medial arteriosclerosis, controlled by using high dosed corticosteroids combined with the usage of warm compresses and transcutaneous electrical nerve stimulation (TENS).[24]

28 Year Old Male

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A 28-year-old male in Saudi Arabia presented with swelling in both of his thighs, despite no past medical history significant of chronic illness, trauma, or surgeries. The individual noted significant pain due to the swelling and reported difficulty walking. As a result of this, the individual was subjected to physical examination, hematological investigations, and a variety of imaging such as a doppler examination, radiograph and MRI. This testing uncovered calcified arteries and a collection of soft tissue near the femoral arteries and veins, which lead to a disruption in the right femoral artery. Extensive analysis of the individual's condition lead medical professionals to the conclusion that Mönckeberg's arteriosclerosis was suspected as the cause for their symptoms. The individual was treated with antibiotics, bed rest, ice packs, as well as anticoagulation therapy. They also received an interventional surgery to help remove the excess fluid from their thighs. After subsequent follow-up, the individual's ability to walk was restored and imaging of their arterial calcification had improved.[18]

Monckeberg’s arteriosclerosis in populations with premature menopause
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A case report describes a 69-year-old female with Monckeberg’s arteriosclerosis affecting her uterine vessels, following long-term endometritis and experiencing premature menopause. [25]

Mönckeberg Medial Arteriosclerosis was found in an Elderly Patient, with Chronic Kidney Disease and Uncontrolled Diabetes, Presenting for a Routine Dental Check-Up
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Mönckeberg medial arteriosclerosis, also known as medial arterial calcinosis, is a condition where the tunica media layer of blood vessels calcifies, visible on plain radiography or sectional tomography, and can sometimes be detected in dental panoramic radiographs. Unlike atherosclerosis, which affects the tunica intima and alters the vessel lumen, this condition is associated with diabetes mellitus or chronic kidney disease, and dental treatment is safe when the patient's diabetes is well-managed.[26]

Epidemiology

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The prevalence of Mönckeberg's arteriosclerosis increases with age and is more frequent in diabetes mellitus, chronic kidney disease, systemic lupus erythematosus, chronic inflammatory conditions, hypervitaminosis D and rare genetic disorders, such as Keutel syndrome.[2] The prevalence of Mönckeberg's arteriosclerosis in the general population has been estimated as <1% on the basis of an ankle brachial pressure index >1.5;[21][27][28] however the validity of this criterion is questionable.[28]

References

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  1. ^ "Mönckeberg arteriosclerosis" at Dorland's Medical Dictionary
  2. ^ a b c d e Micheletti RG, Fishbein GA, Currier JS, Singer EJ, Fishbein MC (August 2008). "Calcification of the internal elastic lamina of coronary arteries". Modern Pathology. 21 (8): 1019–1028. doi:10.1038/modpathol.2008.89. PMC 7029955. PMID 18536656.
  3. ^ synd/4064 at Who Named It?
  4. ^ J. G. Mönckeberg. Über die reine Mediaverkalkung der Extremitätenarterien und ihr Verhalten zur Arteriosklerose. Virchows Archiv für pathologische Anatomie und Physiologie, und für klinische Medicin, Berlin, 1903, 171: 141-167.
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  6. ^ Razzaque MS (2013). "Phosphate toxicity and vascular mineralization". Contributions to Nephrology. 180: 74–85. doi:10.1159/000346784. ISBN 978-3-318-02369-5. PMID 23652551.
  7. ^ a b Steinmann BU, Royce PM (2002). Connective tissue and its heritable disorders: molecular, genetic, and medical aspects. New York: Wiley-Liss. ISBN 978-0-471-25185-9.
  8. ^ Ono K, Tsuchida A, Kawai H, Matsuo H, Wakamatsu R, Maezawa A, et al. (June 2003). "Ankle-brachial blood pressure index predicts all-cause and cardiovascular mortality in hemodialysis patients". Journal of the American Society of Nephrology. 14 (6): 1591–1598. doi:10.1097/01.asn.0000065547.98258.3d. PMID 12761260.
  9. ^ Dao HH, Essalihi R, Bouvet C, Moreau P (May 2005). "Evolution and modulation of age-related medial elastocalcinosis: impact on large artery stiffness and isolated systolic hypertension". Cardiovascular Research. 66 (2): 307–317. doi:10.1016/j.cardiores.2005.01.012. PMID 15820199.
  10. ^ Cuevas Castillo FJ, Sujanani S, Chetram VK, Elfishawi M, Abrudescu A (July 2020). "Monckeberg Medial Calcific Sclerosis of the Temporal Artery Masquerading as Giant Cell Arteritis: Case Reports and Literature Review". Cureus. 12 (7): e9210. doi:10.7759/cureus.9210. PMC 7387070. PMID 32754413.
  11. ^ Shichijo C, Kai K, Jinnouchi K, Nishihara M, Hara H, Aishima S (October 2021). "Intracranial Mönckeberg's Atherosclerosis Is Frequently Found in Autopsy Cases of Advanced Stage Malignancy with Cerebral Infarction". Cancers. 13 (20): 5234. doi:10.3390/cancers13205234. PMC 8534181. PMID 34680388.
  12. ^ a b Amann K (November 2008). "Media calcification and intima calcification are distinct entities in chronic kidney disease". Clinical Journal of the American Society of Nephrology. 3 (6): 1599–1605. doi:10.2215/CJN.02120508. PMID 18815240.
  13. ^ a b McCullough PA, Agrawal V, Danielewicz E, Abela GS (November 2008). "Accelerated atherosclerotic calcification and Monckeberg's sclerosis: a continuum of advanced vascular pathology in chronic kidney disease". Clinical Journal of the American Society of Nephrology. 3 (6): 1585–1598. doi:10.2215/CJN.01930408. PMID 18667741.
  14. ^ Liu Y, Shanahan CM (January 2011). "Signalling pathways and vascular calcification". Frontiers in Bioscience. 16 (4): 1302–1314. doi:10.2741/3790. PMID 21196233.
  15. ^ Couri CE, da Silva GA, Martinez JA, Pereira F, de Paula FJ (December 2005). "Mönckeberg's sclerosis - is the artery the only target of calcification?". BMC Cardiovascular Disorders. 5: 34. doi:10.1186/1471-2261-5-34. PMC 1326214. PMID 16343348.
  16. ^ Tanimura A, McGregor DH, Anderson HC (1986). "Calcification in atherosclerosis. I. Human studies". Journal of Experimental Pathology. 2 (4): 261–273. PMID 2946818.
  17. ^ Tanimura A, McGregor DH, Anderson HC (1986). "Calcification in atherosclerosis. II. Animal studies". Journal of Experimental Pathology. 2 (4): 275–297. PMID 3783282.
  18. ^ a b Odah AM, Khalid MO, Alsaati AA, Alqassab HA, Alkouder GR, Alhejji MH, et al. (April 2023). "Mönckeberg's Disease With Calcified Lower Limb Ischemia in Saudi Arabia: A Rare Case Report and Literature Review". Cureus. 15 (4): e38345. doi:10.7759/cureus.38345. PMC 10229102. PMID 37261176.
  19. ^ Stack A, Sheffield S, Seegobin K, Maharaj S (June 2020). "Mönckeberg medial sclerosis". Cleveland Clinic Journal of Medicine. 87 (7): 396–397. doi:10.3949/ccjm.87a.19085. PMID 32605975.
  20. ^ Hirschl M, Francesconi M, Hirschl MM (1991). "[Moenckeberg media sclerosis: clinical aspects in diabetic patients]". VASA. Zeitschrift Fur Gefasskrankheiten (in German). 20 (3): 216–221. PMID 1950137.
  21. ^ a b Newman AB, Siscovick DS, Manolio TA, Polak J, Fried LP, Borhani NO, et al. (September 1993). "Ankle-arm index as a marker of atherosclerosis in the Cardiovascular Health Study. Cardiovascular Heart Study (CHS) Collaborative Research Group". Circulation. 88 (3): 837–845. doi:10.1161/01.cir.88.3.837. PMID 8353913.
  22. ^ Sato Y, Watanabe R, Uchiyama N, Ozawa N, Takahashi Y, Shirai R, et al. (December 2018). "Inhibitory effects of vasostatin-1 against atherogenesis". Clinical Science. 132 (23): 2493–2507. doi:10.1042/CS20180451. PMID 30401690.
  23. ^ Stack A, Sheffield S, Seegobin K, Maharaj S (June 2020). "Mönckeberg medial sclerosis". Cleveland Clinic Journal of Medicine. 87 (7): 396–397. doi:10.3949/ccjm.87a.19085. PMID 32605975.
  24. ^ Thomas DC, Thomas P, Sivan A, Unnam P, Ajayakumar A, Kumar SS, et al. (June 2021). "Monckeberg's Medial Sclerosis as a Cause for Headache and Facial Pain". Current Pain and Headache Reports. 25 (8): 50. doi:10.1007/s11916-021-00965-0. PMID 34086132.
  25. ^ Mercer VJ, Naseemuddin A, Webb A (December 2021). "Monckeberg's arteriosclerosis: a case report of chronic endometritis presenting as postmenopausal bleeding". Menopause. 29 (2): 247–249. doi:10.1097/GME.0000000000001902. PMID 34905746.
  26. ^ Jensen L, Syed AZ, Odell S, Genung KE, Mupparapu M (July 2023). "Mönckeberg Medial Arteriosclerosis in a Geriatric Patient with Chronic Kidney Disease and Poorly Controlled Diabetes Reporting for a Dental Recall Visit". Dental Clinics of North America. 67 (3): 461–464. doi:10.1016/j.cden.2023.02.019. PMID 37244715.
  27. ^ Meijer WT, Hoes AW, Rutgers D, Bots ML, Hofman A, Grobbee DE (February 1998). "Peripheral arterial disease in the elderly: The Rotterdam Study". Arteriosclerosis, Thrombosis, and Vascular Biology. 18 (2): 185–192. doi:10.1161/01.ATV.18.2.185. hdl:1765/20056. PMID 9484982.
  28. ^ a b Kröger K, Stang A, Kondratieva J, Moebus S, Beck E, Schmermund A, et al. (2006). "Prevalence of peripheral arterial disease - results of the Heinz Nixdorf recall study". European Journal of Epidemiology. 21 (4): 279–285. doi:10.1007/s10654-006-0015-9. PMID 16685578.
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