Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review
Reversible cerebral vasoconstriction syndrome (RCVS) typically manifests as a sudden, severe thunderclap headache due to narrowing of the cerebral arteries. Symptoms usually resolve within three months. An imbalance in cerebral vascular tone, an abnormal endothelial function, and a decreased autoreg...
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| Format: | Article |
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MDPI AG
2023-09-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/13/18/2951 |
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| author | Fahidah Alenzi David P D’Cruz |
| author_facet | Fahidah Alenzi David P D’Cruz |
| author_sort | Fahidah Alenzi |
| collection | DOAJ |
| description | Reversible cerebral vasoconstriction syndrome (RCVS) typically manifests as a sudden, severe thunderclap headache due to narrowing of the cerebral arteries. Symptoms usually resolve within three months. An imbalance in cerebral vascular tone, an abnormal endothelial function, and a decreased autoregulation of cerebral blood flow are thought to be involved in the pathogenesis of RCVS. However, the precise origin of this condition is not yet fully understood. Symptoms of Raynaud’s phenomenon (RP) include vasospasm of arterioles of the digits. The pathophysiology of RP includes interactions between the endothelium, smooth muscle, and autonomic and sensory neurons that innervate arteries to help maintain vasomotor homeostasis. RP may occur before the clinical manifestation of a rheumatic condition. RCVS is rare in patients with autoimmune rheumatic disease. We describe a 54-year-old female who had a history of Raynaud’s phenomenon affecting her fingers and toes since the age of 12 years. The patient was diagnosed with RCVS in 2012. She described RCVS precipitants, including the regular use of cannabis, cocaine, and amphetamine and tobacco smoking. In 2021, she presented with oral ulcers, intermittent swallowing difficulties, and Raynaud’s phenomenon. Clinical examination revealed early sclerodactyly, and abnormal nail-fold capillaroscopy showed multiple giant capillaries, dilated capillary loops, and areas of capillary hemorrhage with capillary drop-out. The investigation revealed positive ANA, strongly positive SRP antibodies, and Ro60 antibodies. Our case report indicates that there may be a correlation between RCVS and Raynaud’s phenomenon, and a potential connection between RCVS and autoimmune rheumatic diseases. Hence, physicians must be aware of the red flags and subtle differences in neurological abnormalities, such as headaches, in patients with autoimmune rheumatic diseases who have an inactive clinical status to improve patient care and outcomes. |
| first_indexed | 2024-03-10T22:52:01Z |
| format | Article |
| id | doaj.art-48d0a8bf783a404fb070adbfe0e58890 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-10T22:52:01Z |
| publishDate | 2023-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-48d0a8bf783a404fb070adbfe0e588902023-11-19T10:13:51ZengMDPI AGDiagnostics2075-44182023-09-011318295110.3390/diagnostics13182951Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature ReviewFahidah Alenzi0David P D’Cruz1Clinical Sciences Department, College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh 11564, Saudi ArabiaLouise Coote Lupus Unit, Guy’s Hospital, Guy’s and St. Thomas’ Hospitals, NHS Foundation Trust, London SE1 9RT, UKReversible cerebral vasoconstriction syndrome (RCVS) typically manifests as a sudden, severe thunderclap headache due to narrowing of the cerebral arteries. Symptoms usually resolve within three months. An imbalance in cerebral vascular tone, an abnormal endothelial function, and a decreased autoregulation of cerebral blood flow are thought to be involved in the pathogenesis of RCVS. However, the precise origin of this condition is not yet fully understood. Symptoms of Raynaud’s phenomenon (RP) include vasospasm of arterioles of the digits. The pathophysiology of RP includes interactions between the endothelium, smooth muscle, and autonomic and sensory neurons that innervate arteries to help maintain vasomotor homeostasis. RP may occur before the clinical manifestation of a rheumatic condition. RCVS is rare in patients with autoimmune rheumatic disease. We describe a 54-year-old female who had a history of Raynaud’s phenomenon affecting her fingers and toes since the age of 12 years. The patient was diagnosed with RCVS in 2012. She described RCVS precipitants, including the regular use of cannabis, cocaine, and amphetamine and tobacco smoking. In 2021, she presented with oral ulcers, intermittent swallowing difficulties, and Raynaud’s phenomenon. Clinical examination revealed early sclerodactyly, and abnormal nail-fold capillaroscopy showed multiple giant capillaries, dilated capillary loops, and areas of capillary hemorrhage with capillary drop-out. The investigation revealed positive ANA, strongly positive SRP antibodies, and Ro60 antibodies. Our case report indicates that there may be a correlation between RCVS and Raynaud’s phenomenon, and a potential connection between RCVS and autoimmune rheumatic diseases. Hence, physicians must be aware of the red flags and subtle differences in neurological abnormalities, such as headaches, in patients with autoimmune rheumatic diseases who have an inactive clinical status to improve patient care and outcomes.https://www.mdpi.com/2075-4418/13/18/2951Raynaud’s phenomenonreversible cerebral vasoconstriction syndromenail-fold capillaroscopyautoimmune rheumatic diseasessystemic lupus erythematosus |
| spellingShingle | Fahidah Alenzi David P D’Cruz Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review Diagnostics Raynaud’s phenomenon reversible cerebral vasoconstriction syndrome nail-fold capillaroscopy autoimmune rheumatic diseases systemic lupus erythematosus |
| title | Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review |
| title_full | Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review |
| title_fullStr | Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review |
| title_full_unstemmed | Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review |
| title_short | Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review |
| title_sort | reversible cerebral vasoconstriction syndrome and raynaud s phenomenon is there a link between the pathogeneses of their underlying complex etiology a case report and literature review |
| topic | Raynaud’s phenomenon reversible cerebral vasoconstriction syndrome nail-fold capillaroscopy autoimmune rheumatic diseases systemic lupus erythematosus |
| url | https://www.mdpi.com/2075-4418/13/18/2951 |
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