Flammer Syndrome

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The Flammer syndrome is a substantially by dysregulation of the blood supply of clinical, so-called vascular dysregulation, labeled complex features. It can manifest itself in numerous symptoms such as cold hands and feet. It is often associated with low blood pressure and, in certain cases, can lead to diseases such as: B. favor ( predispose ) to normal pressure glaucoma . The Flammer syndrome is named after the Swiss ophthalmologist Josef Flammer and has been an established risk factor for certain diseases in medical terminology since 2013.

history

Vasospasm (singular: vasospasm ) have been known for over 100 years, including in the retina of the eye. These are temporary constrictions of arteries or arterioles with the consequence of a temporary insufficient supply of the corresponding organs or parts of organs. Such spasms can occur in a person in different parts of the body; one then speaks of a vasospastic syndrome. It was later recognized that these spasms in the arteries are mostly just part of a much more global dysregulation of the blood vessels. In addition to spasms, these dysregulations also include inadequate, excessive or weak enlargements of arteries, veins and capillaries. The blood vessels of such individuals respond insufficiently or excessively to stimulation. If this is not due to other diseases, but rather to a predisposition, it is referred to as primary vascular dysregulation (PVD). Interestingly, PVD is almost always linked to other vascular and non-vascular symptoms and signs. This entire complex (PVD and accompanying symptoms) is known today as Flammer's syndrome.

Symptoms

The core symptoms of Flammer's syndrome result from the regulatory disturbance of the blood supply, whereby the symptoms depend on the affected organ. Cold hands or feet, low blood pressure, occasionally white and red spots on the face or neck and migraine-like symptoms or a feeling of pressure behind the upper eyelid are common. In addition, there are also symptoms that are not directly related to the dysregulation of the blood vessels, such as: prolonged time to fall asleep, decreased thirst, high sensitivity not only to cold, but also to smells, vibrations, psychological stress or certain medications (e.g. calcium antagonists , Beta blockers ) etc. Pain and muscle tension are common. People with Flammer Syndrome are usually very precise and highly motivated and successful in their professional lives.

Scientific studies have shown that many patients with glaucoma ( glaucoma ) is caused by a circulatory disorder of the optic nerve and retina is located. Many of these patients with normal pressure glaucoma have a basic constitution that corresponds to Flammer's syndrome. Some of its characteristics were previously grouped under the term "Primary Vascular Dysregulation Syndrome".

Signs of Flammer Syndrome can include:

  • cold hands and / or feet
  • arterial hypotension
  • low body mass index
  • decreased feeling of thirst
  • Extension of sleep time
  • increased sensitivity: sensitivity to pain, perception of smell, sensitivity to certain drugs
  • migraine
  • Tinnitus
  • reversible patchy white or red discoloration of the skin.

People with Flammer Syndrome typically react excessively to psychological or physical stimuli such as stress or exposure to cold. Flammer's syndrome occurs more frequently in the following groups of people: women versus men, Asians versus Caucasians and among academics versus workers, people with indoor jobs versus people with outdoor jobs. These people are mostly highly motivated in their professional life and need longer to fall asleep.

Disease value

Having Flammer Syndrome does not necessarily mean being ill - on the contrary: most of those affected are and will remain healthy. Certain diseases such as B. arteriosclerosis and its consequences are probably even rarer. But other diseases are more common. The best known is the risk of normal pressure glaucoma . If glaucoma damage occurs despite normal intraocular pressure, or if glaucoma damage progresses despite normalized intraocular pressure, then Flammer syndrome is often present. In eyes with glaucoma in Flammer's syndrome, the pressure in the veins of the retina is often increased. Flammer's syndrome can also promote the development of other eye diseases, including vascular occlusions (especially retinal vein occlusions) in relatively young people or central serous retinopathy . Muscle cramps and tension are common. Tinnitus and occasionally hearing loss can occur. There is evidence that Flammer's syndrome appears to be more common in patients with retinopathia pigmentosa and that the vascular factor endothelin-1 may play a role here. An altered gene expression in the lymphocytes has also been described in people with Flammer syndrome .

Beyond the tendency of people with Flammer's syndrome to develop these eye disorders, associations with general ailments have recently been described. In a study with 58 multiple sclerosis patients and 259 healthy control persons, six of fifteen symptoms of Flammer's syndrome were reported statistically significantly more frequently by MS patients; seven other symptoms occurred more frequently in those affected, without the difference to the control group being statistically significant reached. A similarly noticeable accumulation of signs of Flammer's syndrome was found in questioning patients with breast cancer . Eleven of the 15 symptoms asked were recorded by the patients far more often than by healthy women of the same age, especially a feeling of cold when sitting quietly, cold extremities, low blood pressure, headache, tinnitus, skin spots. Two subgroups of patients with breast cancer, those with Her2 status (human epidermal growth factor receptor type 2) and those with triple-negative carcinoma, reported that they were very slim in their youth - one of the most important characteristics of the Flammer syndrome in association with impaired thermoregulation and low blood pressure.

Risk factors

People with Flammer Syndrome almost always have ancestors who suffered from the same thing. So a genetic predisposition is obvious. It is significantly more common in women than in men, and symptoms increase during puberty and decrease with age, in women v. a. after menopause. So the hormones probably play a role. Flammer's syndrome is also more common in academics than craftsmen and more common in people who work indoors than in people who work outdoors. The light may play a role. Furthermore, the syndrome is more common in people with a low body mass index (BMI) than in people with a high BMI. If there is a corresponding predisposition, certain trigger factors can provoke or worsen the symptoms. These triggers include: cold, mechanical or emotional stress, food abstinence, rapid ascent to greater heights (altitude sickness) or certain medications such as B. Adrenaline. A migraine can also cause or worsen the symptoms. People with Flammer Syndrome are usually very flexible. If the sport is exaggerated (e.g. jogging too intensely) then the symptoms can be increased.

diagnosis

The diagnosis or at least the suspicion of Flammer's syndrome is made primarily on the basis of the anamnesis with the typical features. If necessary and desired, the diagnosis can be confirmed with tests. One method is nail fold capillary microscopy. In this examination method, the smallest blood vessels (capillaries) in the fingers show excessive constriction (vasoconstriction) as a reaction to cold stimulation. Another possibility is the retinal vascular analysis : If the retina is stimulated with flicker light, the arteries and veins expand in healthy people. This response is diminished or absent in people with Flammer Syndrome. Laboratory tests such as the analysis of gene expression in the lymphocytes offer further possibilities.

therapy

Flammer syndrome does not require therapy as long as those affected do not suffer from their symptoms or pathological sequelae occur. Treatment is based on three pillars: a) lifestyle interventions, b) diet, and c) medication. Lifestyle includes regular sleep, weight stabilization (in the sense of not being underweight), avoiding fasting periods or avoiding known trigger factors such as B. Cold. Regular physical exercise is beneficial, but unfavorable for extreme sports. The diet should be as antioxidant as possible. These include green tea, black filter coffee, red wine, blue berries and fruits etc. Omega-3 fats, v. a. in the form of fish improve the regulation of blood circulation. If the blood pressure is low, the salt intake should be increased. In the case of drugs, those that can lead to vascular constriction should be avoided. If the blood pressure is too low, sleeping pills should also be used carefully. Magnesium and low-dose calcium channel blockers can help against vascular dysregulations.

Medical treatment is necessary if, for example, normal pressure glaucoma is present. In these cases, in addition to ophthalmological glaucoma therapy, the patient's blood pressure, which is almost always too low, should be adjusted. With dietary measures, for example a high-salt diet, occasionally also with low-dose mineralocorticoid steroids , one tries to reduce a drop in blood pressure - especially during sleep - as this can permanently damage the optic nerve head. Furthermore, the vascular dysregulation in these people is reduced by high-dose magnesium and / or very low-dose calcium channel blockers . Then the increased retinal venous pressure usually also decreases.

literature

  • Josef Flammer, Katarzyna Konieczka: The discovery of Flammer syndrome: a historical and personal perspective. EPMA J. 2017 May 22; 8 (2): 75-97.
  • Josef Flammer, Katarzyna Konieczka: Phenomenology and clinical significance of Flammer's syndrome. Ophthalmic Clinical Monthly Sheets 2016; 233: 1331-1336.
  • Maneli Mozaffarieh, Josef Flammer: Ocular blood flow and glaucomatous optic neuropathy. Springer, Berlin 2009, ISBN 978-3-540-69442-7 .
  • M. Mozaffarieh, J. Flammer: New insights in the pathogenesis and treatment of normal tension glaucoma. In: Current opinion in pharmacology. Volume 13, Number 1, February 2013, pp. 43-49. doi: 10.1016 / j.coph.2012.10.001 . PMID 23092679 . (Review).
  • L. Fang, S. Turtschi, M. Mozaffarieh: The effect of nifedipine on retinal venous pressure of glaucoma patients with the Flammer syndrome. In: Graefes Archive for Clinical and Experimental Ophthalmology 2015; 253: 935-939
  • Ronald D. Barley: Eye disorders with tinnitus and cold extremities. Flammer's syndrome describes the pathogenesis of a variant of glaucoma in which vascular dysregulations not only dominate in the eye. In: Deutsches Ärzteblatt. February 21, 2014; Pp. A308-A309. Online version
  • Ronald D. Gerste : Glaucoma Meeting 2013: Challenges in Glaucoma. In: ophta. 2014, pp. 88–94.
  • K. Konieczka, S. Fränkl et al: Unstable Oxygen Supply and Glaucoma. In: Clinical monthly sheets for ophthalmology. 231, 2014, pp. 121-126, doi: 10.1055 / s-0033-1360242 .

Web links

Individual evidence

  1. ↑ J. Flammer, K. Konieczka, AJ Flammer: The primary vascular dysregulation syndrome: implications for eye diseases. In: The EPMA journal. Volume 4, number 1, 2013, p. 14, ISSN  1878-5077 . doi: 10.1186 / 1878-5085-4-14 . PMID 23742177 . PMC 3693953 (free full text).
  2. ↑ RD Barley: Eye disorders with tinnitus and cold extremities. In: Deutsches Ärzteblatt. February 21, 2014; P. A308 – A309 PDF version
  3. ↑ Konieczka K, Ritch R, Traverso CE, Kim DM, Kook MS, Gallino A, Golubnitschaja O, Erb C, Reitsamer HA, Kida T, Kurysheva N, Yao K. Flammer syndrome. EPMA J. 2014 Jul 8; 5 (1): 11. doi: 10.1186 / 1878-5085-5-11
  4. ↑ K. Konieczka, S. Fränkl: Primary vascular dysregulation and glaucoma (Primary Vascular Dysregulation and Glaucoma). In: Journal of Practical Ophthalmology. 2013; 34, pp. 207-215.
  5. ↑ K. Konieczka, C. Erb: Diseases potentially related to Flammer syndrome, EPMA Journal (2017) 8: 327-332
  6. ↑ Lei Fang, Michael Baertschie, Manneli Mozaffarieh: The effect of flammer-syndrome on venous retinal pressure, BMC Ophthalmology 2014, 14: 121
  7. ^ Katarzyna Konieczka: Flammer syndrome. In: The EPMA Journal. 2014; 5, p. 11.
  8. ↑ MG Todorova et al .: Endothelin-1 Plasma Levels in Patients with Both Retinitis Pigmentosa and Flammer Syndrome. Klin monthly bl Augenheilkd 2015; 232: 514-518
  9. ↑ Konieczka K, Flammer AJ, Todorova M, Meyer P, Flammer J. Retinitis pigmentosa and ocular blood flow. EPMA J. 2012 Dec 3; 3 (1): 17. doi: 10.1186 / 1878-5085-3-17
  10. ^ Konieczka K, Koch S, Schoetzau A, Todorova MG. Increased Prevalence of Flammer Syndrome in Patients with Retinitis Pigmentosa. Klin Monbl Augenheilkd. 2016 Apr; 233 (4): 448-52. doi: 10.1055 / s-0041-111802
  11. ↑ Fraenkl SA, Golubnitschaja O, Yeghiazaryan K, Orgül S, Flammer J: Differences in gene expression in lymphocytes of patients with high-tension, PEX, and normal-tension glaucoma and in healthy subjects. Eur J Ophthalmol. 2013; 23: 841-849
  12. ↑ Konieczka K, Koch S, Binggeli T, Schoetzau A, Kesselring J: Multiple sclerosis and primary vascular dysregulation (Flammer syndrome). EPMA Journal 2016 Jun 15; 7:13
  13. ↑ Zubor P, Gondova A, Polivka J Jr, Kasajova P, Konieczka K, Danko J, Golubnitschaja O: Breast cancer and Flammer syndrome: any symptoms in common for prediction, prevention and personalized medical approach? EPMA Journal 2017 Apr 10; 8 (2): 129-140.
  14. ^ Katarzyna Konieczka: Endothelial dysfunction in glaucoma. In: Ophthalmological News. July 2014.
  15. ↑ K. Konieczka, S. Fränkl u. a .: Unstable oxygen supply and glaucoma. In: Clinical monthly sheets for ophthalmology. Volume 231, Number 2, February 2014, pp. 121-126. doi: 10.1055 / s-0033-1360242 . PMID 24532398 .
  16. ↑ Mozaffarieh M, Flammer J. Is there more to glaucoma treatment than lowering IOP? Surv ophthalmol. 2007 Nov; 52 Suppl 2: S174-9