Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis
Benign joint hypermobility syndrome (BJHS) and postural orthostatic tachycardia syndrome (POTS) are two common conditions which are frequently overlooked. While patients with BJHS are known to attend rheumatology, orthopedic, and medical outpatient departments for years with polyarthralgia; POTS is...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2017-01-01
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| Series: | Medical Journal of Dr. D.Y. Patil University |
| Subjects: | |
| Online Access: | http://www.mjdrdypu.org/article.asp?issn=0975-2870;year=2017;volume=10;issue=2;spage=175;epage=178;aulast=Kaur |
| _version_ | 1818989817533300736 |
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| author | Navjyot Kaur V A Arun Shavana Rana V K Sashindran |
| author_facet | Navjyot Kaur V A Arun Shavana Rana V K Sashindran |
| author_sort | Navjyot Kaur |
| collection | DOAJ |
| description | Benign joint hypermobility syndrome (BJHS) and postural orthostatic tachycardia syndrome (POTS) are two common conditions which are frequently overlooked. While patients with BJHS are known to attend rheumatology, orthopedic, and medical outpatient departments for years with polyarthralgia; POTS is commonly misdiagnosed as anxiety neurosis or panic attack. Described first in 1940, POTS is one of the common causes of orthostatic symptoms in females. POTS is defined as orthostatic intolerance associated with tachycardia exceeding 120 beats/min (bpm) or an increase in the heart rate (HR) of 30 bpm from baseline within 10 min of changing the posture from a lying to standing position, in the absence of long-term chronic diseases and medications that affect the autonomic or vascular tone. Classified as primary and secondary, the underlying pathophysiological mechanism is assumed to be a failure of peripheral vascular resistance to increase sufficiently in response to orthostatic stress, and consequently, venous pooling occurs in the legs resulting in decreased venous return to the heart. This is compensated by an increase in HR and inotropy. We present a case of BJHS, who reported to us with recurrent episodes of syncope and presyncope and was diagnosed to have POTS secondary to his hypermobility syndrome. Although the tilt-table test is the gold standard for diagnosis of POTS, this case highlights the importance of bedside tests in evaluation of orthostatic symptoms and in diagnosis of relatively common but frequently overlooked syndrome. |
| first_indexed | 2024-12-20T19:44:30Z |
| format | Article |
| id | doaj.art-383a1287034c4c58aeacedcd0ff24b4f |
| institution | Directory Open Access Journal |
| issn | 0975-2870 |
| language | English |
| last_indexed | 2024-12-20T19:44:30Z |
| publishDate | 2017-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Medical Journal of Dr. D.Y. Patil University |
| spelling | doaj.art-383a1287034c4c58aeacedcd0ff24b4f2022-12-21T19:28:27ZengWolters Kluwer Medknow PublicationsMedical Journal of Dr. D.Y. Patil University0975-28702017-01-0110217517810.4103/0975-2870.202108Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosisNavjyot KaurV A ArunShavana RanaV K SashindranBenign joint hypermobility syndrome (BJHS) and postural orthostatic tachycardia syndrome (POTS) are two common conditions which are frequently overlooked. While patients with BJHS are known to attend rheumatology, orthopedic, and medical outpatient departments for years with polyarthralgia; POTS is commonly misdiagnosed as anxiety neurosis or panic attack. Described first in 1940, POTS is one of the common causes of orthostatic symptoms in females. POTS is defined as orthostatic intolerance associated with tachycardia exceeding 120 beats/min (bpm) or an increase in the heart rate (HR) of 30 bpm from baseline within 10 min of changing the posture from a lying to standing position, in the absence of long-term chronic diseases and medications that affect the autonomic or vascular tone. Classified as primary and secondary, the underlying pathophysiological mechanism is assumed to be a failure of peripheral vascular resistance to increase sufficiently in response to orthostatic stress, and consequently, venous pooling occurs in the legs resulting in decreased venous return to the heart. This is compensated by an increase in HR and inotropy. We present a case of BJHS, who reported to us with recurrent episodes of syncope and presyncope and was diagnosed to have POTS secondary to his hypermobility syndrome. Although the tilt-table test is the gold standard for diagnosis of POTS, this case highlights the importance of bedside tests in evaluation of orthostatic symptoms and in diagnosis of relatively common but frequently overlooked syndrome.http://www.mjdrdypu.org/article.asp?issn=0975-2870;year=2017;volume=10;issue=2;spage=175;epage=178;aulast=KaurBenign hypermobility syndromepostural orthostatic tachycardia syndrometilt-table test |
| spellingShingle | Navjyot Kaur V A Arun Shavana Rana V K Sashindran Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis Medical Journal of Dr. D.Y. Patil University Benign hypermobility syndrome postural orthostatic tachycardia syndrome tilt-table test |
| title | Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis |
| title_full | Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis |
| title_fullStr | Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis |
| title_full_unstemmed | Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis |
| title_short | Benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis |
| title_sort | benign joint hypermobility syndrome with postural orthostatic tachycardia syndrome and acrocyanosis |
| topic | Benign hypermobility syndrome postural orthostatic tachycardia syndrome tilt-table test |
| url | http://www.mjdrdypu.org/article.asp?issn=0975-2870;year=2017;volume=10;issue=2;spage=175;epage=178;aulast=Kaur |
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