Vasculitides of large vessels.

Vasculitides are inflammatory diseases affecting large, medium or small sized vessels, caused by various immunological processes and possibly triggered by infectious agents. Vascular medicine is particularly concerned with vasculitides affecting large vessels: Takayasu arteritis (TA) and giant cell arteritis (GCA, temporal arteritis), which are determined by a similar special immunopathology, further aortitis and Behçet disease (BD). Recent progress in vascular imaging allows the detection of vessel alterations at an earlier stage and a better control of the course of disease. While cranial symptoms prevail in most elder GCA patients, involvement of larger vessels is increasingly perceived. This especially concerns a subgroup called large vessel GCA in which inflammatory obstructions mainly affect the proximal arm arteries or aorta, predominantly in women of the 6th and 7th decade. Initial therapy is based on corticosteroids. Of other immunosuppressive drugs only methotrexate might be considered in GCA. Disease activity has to be monitored both by laboratory parameters and vascular imaging. Aortitis due to GCA may result in late aneurysms even in patients with good response to medical treatment. Aneurysm development may also be the result of isolated necrotizing aortitis. In Behçet disease, vessel involvement is seen in about 20 % of patients. Regarding arteries, aneurysm formation is more frequent than obstruction. Venous thrombosis is often extended and of central location but rarely leads to pulmonary embolism. Although a specific therapy is not established, immunosuppression seems to be of greatest importance and anticoagulation may be withheld.

[Large vessel vasculitis]. / Vaskulitiden der grossen Gefässe.

Large vessel vasculitis is characterized by local inflammation of the vessel wall of the aorta and its first and second degree branches. Giant cells are found regularly. The process leads to stenoses and occlusions as well as to aneurysmatic dilatation. Recent progress in vascular imaging (colour Doppler ultrasound, MRI and PET) allows to detect Takayasu arteritis at an earlier stage and to reveal the involvement of larger arteries in temporal arteritis (giant cell arteritis, GCA). With increasing frequency, a subtype of predominantly female and less aged GCA patients without cranial symptoms is noticed in whom obstructions are prevailing in the proximal arm vessels or in the aorta. The latter may be affected exclusively. Initial therapy is based on corticosteroids. In GCA, methotrexate hitherto is the only other immunosuppressive drug that has proved to be helpful. Disease activity has to be controlled both by laboratory parameters and vascular imaging.

Intra-arterial and intravenous administration of prostaglandin E1 cause different changes to skin microcirculation in patients with peripheral arterial occlusive disease.

BACKGROUND: Disturbed skin microcirculation in patients with peripheral arterial occlusive disease (PAOD) might be affected by prostaglandin E1 (PGE1). The purpose of this study was to investigate local skin perfusion at the forefoot and toe of PAOD patients during different modes of administration of PGE1. PATIENTS AND METHODS: In 17 patients with severe PAOD local intradermal haemoglobin oxygen saturation (SHB) and dermal vascular reserve capacity, transcutaneous oxygen pressure (tcPO2) and laser Doppler flux (LDF) were determined during intra-arterial (1.5 ng/kg body weight/min) and intravenous (6 ng/kg body weight/min) infusion of PGE1. RESULTS: SHB significantly increased at both locations during intravenous PGE1; intra-arterial administration significantly increased SHB in the skin of the forefoot only. Reserve capacity increased during intravenous administration and was markedly reduced during intra-arterial infusion. No significant changes were found regarding tcPO2 and LDF. CONCLUSIONS: These data suggest different therapeutic mechanisms for intra-arterial and intravenous administered of PGE1.

Circadian variation of skin perfusion in arterial occlusive disease.

BACKGROUND: Many physiological processes are subjected to circadian variation. We tested if circadian changes of skin perfusion can be detected by measuring laser Doppler flux (LDF) in the foot and if such variations are different in patients with peripheral arterial occlusive disease (PAOD). PATIENTS AND METHODS: LDF was recorded hourly during a 24-hour period in 10 healthy subjects and in 19 PAOD patients, 9 presenting with claudication (stage II according to Fontaine, ankle artery pressures (AP) 80 +/- 11 mmHg) and 10 with skin necrosis (stage IV, AP 42 +/- 29 mmHg). RESULTS: Circadian variability of LDF was significantly reduced in stage IV patients compared both with stage II patients and controls (p < 0.01). An increase in LDF could be observed early in the afternoon and, more pronounced, during the night, occurring to the same extent in healthy and PAOD patients in stage II (p < 0.001). In stage IV the increase in LDF was not significant and a decrease was observed in two patients. LDF and local skin temperature were correlated in controls and stage II patients (mean r = 0.69 +/- 0.12 and 0.7 +/- 0.14, respectively), but not in stage IV (mean r = 0.23 +/- 0.18). A further limitation of LDF variability and nocturnal increase was seen in the presence of diabetes. CONCLUSION: Circadian variations of skin blood flow are comparable in controls and patients with claudication but markedly reduced in patients with severe PAOD presenting with ulcers, in whom skin perfusion seems uncoupled from temperature regulation, possibly due to local factors causing continuous arteriolar vasodilation.

Changes of dermal haemoglobin oxygenation and concentration in healthy subjects and in patients with peripheral arterial occlusive disease during and after acute ischemia.

BACKGROUND: Oxygen saturation (SHB) and concentration (CHB) of dermal haemoglobin play an important role in the nutrition of the skin. In patients with severe peripheral arterial occlusive disease (PAOD) they are reduced at the forefoot. We investigated the changes of the named parameters during an acute ischemia and during reactive hyperemia using an occlusion test. PATIENTS AND METHODS: Dermal reflection spectra were conducted from the forefoot of 11 healthy subjects and 39 patients with peripheral arterial occlusive disease of various stages at rest, during, and after an arterial occlusion at the thigh. Using a multicomponent analysis SHB and CHB were calculated iteratively. The halftime of the post-occlusive increase of SHB (post-occlusive recovery halftime PORHT) and the relation of post- to pre-occlusive CHB (post-occlusive reactive hyperemia PORH) were calculated to estimate the dynamic of the post-occlusive inflow of blood. TcPO2 (37 degrees C) was additionally determined at the forefoot. RESULTS: In patients from stage III and IV all resting values were lower compared to controls. TcPO2 decreased in all persons to 0 mm Hg during the occlusion, SHB was between 0 and 10%, while CHB remained unchanged. In healthy subjects and in patients with claudication the post-occlusive values exceeded the preocclusive ones, while in some patients with higher stages of the disease this could not be found. There was a marked, on the stage of the disease depending difference in the post-occlusive course between controls and patients: in healthy subjects it took only 5.5 +/- 4.8 s until SHB reached half of the maximum value, in patients from the stage IIa group 26.8 +/- 16.9 s (p < 0.001) was measured while in stage III and IV the value was 132 +/- 75 s (p < 0.001). CONCLUSIONS: During ischemia a decrease of the haemoglobin oxygen saturation (SHB) and tcPO2 was found. Haemoglobin concentration (CHB) remained constant, hence a significant shift of blood from skin to muscle did not take place. PORHT was selectively dependent on the stage of the disease. The occlusion manoeuvre enhances the significance of reflection photometry in PAOD.

Nailfold capillaroscopy and laser Doppler fluxmetry for evaluation of Raynaud's phenomenon: how valid is the local cooling test?

BACKGROUND: Since there is a need for an objective parameter of microcirculation in follow-up of patients with Raynaud's syndrome we evaluated the reproducibility of the reaction to a cold exposure test with nailfold capillaroscopy and laser Doppler fluxmetry during a winter period. PATIENTS AND METHODS: 10 healthy subjects and 16 patients with primary Raynaud's syndrome were evaluated with nailfold capillaroscopy and laser Doppler fluxmetry of the finger tips during and after a standardized cold exposure stress test. The measurements were repeated in the controls within 2 weeks, in the patient population twice within 24 weeks. RESULTS: In the healthy controls the percentage of capillaries with flow stop was stable (r = 0.785) and the duration of flow stop was reproducible (r = 0.993). Both parameters were significantly lower in controls than in the patients. The flow stop durations in patients varied intra-individually to a great extent between the different weeks without any significant correlation. For all calculated laser Doppler perfusion and time parameters we did not find any significant differences between controls and patients nor any consistent correlations in the intra-individual comparison within the different weeks for both, controls and patients. CONCLUSION: Nailfold capillaroscopy during cold exposure is able to discriminate between healthy persons and patients with primary Raynaud's syndrome, but seems to be of minor value for follow-up evaluation of patients because of intraindividual variations. Laser Doppler fluxmetry is invalid for both purposes when using the applied cold exposure test.

Vital capillary microscopy of skin areas at the forefoot of diabetic patients using intraarterial injection of Na-fluorescein.

A modified technique of vital capillary microscopy with intraarterial application of Na-fluorescein has been introduced in the study of nutritional skin microcirculation to assess skin microcirculation of different diabetic patients, comprising one group without neurocutaneous complications (group 2; n = 9), one suffering only from neuropathy (group 3; n = 9) and one with trophic skin lesions in the contralateral foot (group 4; n = 8), all without macroangiopathy, compared to healthy controls (group 1; n = 9). Femoroarterial injection of small boli (10 mg) of Na-fluorescein allowed repeated investigation of the dye appearance times (AT) and capillary-filling times of forefoot skin capillaries within small periods of time before, during and after reactive hyperemia. At rest, AT was significantly shorter in patients of group 4 (16.8 +/- 4.4 s; p < 0.05) compared with groups 1-3 (34.3 +/- 12.8; 31.7 +/- 11.7 and 35.9 +/- 15.3 s). Fifteen seconds after the end of arterial occlusion, dye propagation to the skin was markedly accelerated in groups 1-3 (19.8 +/- 14.0; 14.4 +/- 7.6 and 18.7 +/- 10.6 s, respectively; p < 0.001), but prolonged in group 4 (18.4 +/- 7.4 s). After 10 min, the values at rest were reestablished. No differences between the four groups were found concerning capillary density and morphology. It is concluded that the development of skin lesions in diabetic patients without significant macroangiopathy may be favored by hyperperfusion and impaired vasoregulation. Intraarterial dye injection presents a valuable tool to assess dynamic alterations of the microcirculation at the level of skin capillaries.

Treatment of primary Raynaud's syndrome with traditional Chinese acupuncture.

OBJECTIVE: Evaluation of the effects of a standardized acupuncture treatment in primary Raynaud's syndrome. DESIGN: A controlled randomized prospective study. SETTING: A winter period of 23 weeks, angiological clinic of Hannover Medical School. SUBJECTS: Thirty-three patients with primary Raynaud's syndrome (16 control, 17 treatment). INTERVENTIONS: The patients of the treatment group were given seven acupuncture treatments during the weeks 10 and 11 of the observation period. MAIN OUTCOME MEASURES: All patients kept a diary throughout the entire observation period noting daily frequency, duration and severity of their vasospastic attacks. A local cooling test combined with nailfold capillaroscopy was performed for all patients at baseline (week 1) and in weeks 12 and 23, recording flowstop reactions of the nailfold capillaries. RESULTS: The treated patients showed a significant decrease in the frequency of attacks from 1.4 day-1 to 0.6 day-1, P < 0.01 (control 1.6 to 1.2, P = 0.08). The overall reduction of attacks was 63% (control 27%, P = 0.03). The mean duration of the capillary flowstop reaction decreased from 71 to 24 s (week 1 vs. week 12, P = 0.001) and 38 s (week 1 vs. week 23, P = 0.02) respectively. In the control group the changes were not significant. CONCLUSIONS: These findings suggest that traditional Chinese acupuncture is a reasonable alternative in treating patients with primary Raynaud's syndrome.

[Standardization of nailfold capillary microscopy in routine diagnosis]. / Standardisierung der Nagelfalz-Kapillarmikroskopie in der Routinediagnostik.

BACKGROUND: Nailfold capillary microscopy is scientifically established and recognized as a diagnostic tool in clinical routine. It is, however, practiced in only a few centres. METHODS: But since a more widespread use of the method is to be expected for the near future, a consensus-meeting was held, where all German-speaking clinicians and scientists, involved in capillary microscopy, were invited. Here, the technical procedure and the most important morphological parameters were evaluated and defined according to practical clinical aspects. RESULTS AND CONCLUSION: The main task of the consensus-meeting was to develop a standard evaluation sheet, accompanied by an explanation sheet. This standard evaluation sheet focuses on semiquantitative registration of morphological parameters like density, dilation, avascular fields, perivascular edema, microbleeding, torsion, atypical capillaries and giant capillaries. This standard evaluation sheet is to be validated by determining the inter-observer-variance. Later-on an illustrated practical guideline will be published.

Non-invasive determination of dermal hemoglobin oxygenation and concentration in patients with peripheral arterial occlusive disease.

BACKGROUND: Oxygen saturation (SHB) and concentration (CHB) of dermal hemoglobin are important parameters for the supply of the skin. They may be non-invasively assessed by means of reflection photometry as hemoglobin is predominantly contributing to the skin reflection spectrum. METHODS AND RESULTS: Dermal reflection spectra from the middle of the forefoot and from the tip of the toe of 20 healthy subjects and 61 patients suffering from peripheral arterial occlusive disease (PAOD) were recorded and compared to transcutaneous oxygen pressure (tcPO2). SHB, and tcPO2 were significantly higher in controls: compared to patients (ankle pressure (AP) > 50 mmHg) SHB in the skin of the forefoot was 63.8 vs. 54.5% (p < 0.05), CHB was 0.89 vs. 0.62 aU (p < 0.01). In the great toe SHB was 92.9 vs. 59.9% (p < 0.001), CHB was 2.21 vs. 0.92 aU (p < 0.001). Heating the skin of the forefoot to 44 degrees C yields local dermal vascular reserve capacity: SHB and CHB increased in all healthy subjects (96.4%; 1.44 aU, respectively). SHB decreased at the forefoot in 14 and at the toe in 18 of 21 patients with AP < 50 mmHg. At the toe decrease was found in 50 of 61 patients (sensitivity 82%, specificity 100%). CONCLUSIONS: Local vascular reserve capacity of dermal perfusion is reduced in patients with PAOD, depending on AP. It should be determined preferably in patients with severe PAOD.

A new method for the assessment of tissue hemoglobin oxygenation in patients with chronic venous insufficiency.

Venous incompetence often leads to ulceration of the skin of the lower limb due to a disturbance of skin microcirculation. We investigated 10 healthy subjects and 18 patients with chronic venous insufficiency by means of a fast scanning reflection photometer and determined noninvasively the hemoglobin oxygen saturation (S(HB)) and the relative concentration of hemoglobin (C(HB)) in the skin of the forefoot and at the calf, 10 cm proximal of the inner malleolus during positional changes. In supine position no significant differences were found. During sitting and standing S(HB) dropped and was significantly lower in patients at both locations (calf, standing, patients 22.2 +/- 10.6% vs controls 48.2 +/- 13.5%, P < 0.001). C(HB) increased in patients during standing from 0.71 to 2.13 +/- 0.33 aU (in controls from 0.69 to 1.49 +/- 0.28 aU, P < 0.001). Activation of the muscle pump decreased C(HB) and increased S(HB) in patients and in controls. After the movements were stopped, S(HB) decreased again and fell to the initial low values eight times faster in patients (7.4 +/- 5.3 vs 57.5 +/- 19.6 sec, P < 0.001). C(HB) increased four times faster in patients (7.2 +/- 6.1 vs 29.9 +/- 13.6 sec). In sitting and standing positions hemoglobin oxygenation in the skin of the lower limb was markedly reduced in patients, but normal perfusion conditions were restored in supine position, indicating reversibility of the changes. Reflection oxymetry seems a valuable tool for the assessment of chronic venous insufficiency. The substantially reduced oxygenation of dermal hemoglobin in standing patients may contribute to the development of venous ulcers.

Orthostatic vasoconstrictor response in patients with occlusive arterial disease assessed by laser Doppler flux and transcutaneous oximetry.

Posturally induced microvascular constriction normally causes a decrease of transcutaneous oxygen pressure (tcPO2) and laser Doppler flux (LDF) measured on the forefoot at 37 degrees C. The authors used both methods to assess the vasoconstrictor response (VCR) in 31 patients with various degrees of peripheral arterial occlusive disease (PAOD) and analyzed factors that could have influenced the response. Disturbed VCR was indicated by a signal increase following leg dependency, which occurred significantly more often in tcPO2 than in LDF measurements (69% vs 32%, P < 0.001). Correspondingly the median sitting/supine ratio was 2.4 for tcPO2 and 0.7 for LDF (P < 0.0001). Age and clinical stage had no influence on the VCR. With ankle artery pressures below 50 mmHg an increase of LDF was more probable. TcPO2 predominantly increased with ankle artery pressure up to 100 mmHg, though the sitting/supine ratio of tcPO2 was correlated with ankle artery pressure. In nondiabetics the response of tcPO2 but not of LDF was influenced by the values at rest. Differences between the two methods may be explained in part by their different sample volumes. The authors assume that tcPO2 is predominantly monitoring a local myogenic response while LDF is reflecting venoarteriolar response.

[Cystic degeneration of the adventitia of the popliteal artery after PTA of the superficial femoral artery]. / Zystische Adventitiadegeneration der A. poplitea bei Zustand nach PTA der A. femoralis superficialis.

Cystic adventitial degeneration is rare. Most frequently it occurs in the popliteal artery, occasionally it may be found in other arteries and even in veins. It is always localized in joint-adjacent vessel areas and appears to originate in ectopic synovial tissue occasionally with a stemlike connection with the adjacent joint. The diagnosis of a cystic adventitial degeneration should be considered in cases of isolated stenosis or occlusion of the popliteal artery. Realtime ultrasound or computerized tomography may help to establish the diagnosis. The treatment of choice is surgival removal of the cysts or reconstruction with an autologous vein interponate. A rare case of simultaneous occurrence of cystic adventitial degeneration and arteriosclerotic occlusive disease is described.