Microangiopathy in Rheumatic Diseases

Topic Information

Dear Colleagues,

Raynaud’s phenomenon (RP) is a frequent symptom in rheumatic diseases. The frequency of RP is highest in systemic sclerosis (SSc), in which it occurs in more than 90% of cases as it frequently precedes other symptoms of the diseases. Endothelial dysfunction is a key initial process in the pathogenesis of SSc. In patients with undifferentiated connective tissue disease, it is observed in approximately half of the patients, in systemic lupus erythematosus—in 1/3 of cases. An easy and accessible method for evaluation of the microvascular pathology is capillaroscopy. The characteristic pathological capillaroscopic changes (“scleroderma” type capillaroscopic pattern) are observed in more than 90% of SSc patients and are included in the current classification criteria for the disease. Specific autoantibodies are also used to support the early diagnosis of the disease. Analogous capillaroscopic changes are observed also in other rheumatic diseases such as undifferentiated connective tissue disease, overlap syndromes, systemic lupus erythematosus, etc. (i.e., “scleroderma-like” capillaroscopic changes). As capillaroscopic features of microangiopathy are of crucial importance for early diagnosis of rheumatic diseases with peripheral vascular syndrome, important topic of research is studying the characteristics of early microangiopathy. In addition, differentiation between “scleroderma” and “scleroderma-like” capillaroscopic pattern is an interesting issue in the research agenda. Presence of association between capillaroscopic features of microangiopathy and autoantibodies is a question that is insufficiently studied in the contemporary rheumatology. The current Special Issue discusses different aspects of microangiopathy in rheumatic diseases whose better understanding would facilitate early diagnosis of microangiopathy and support improvement of knowledge of disease pathogenesis.

Dr. Sevdalina Lambova
Dr. Erik Lubberts
Topic Editors

Keywords

Participating Journals

Journal Name	Impact Factor	CiteScore	Launched Year	First Decision (median)	APC
Life life	3.2	4.3	2011	17.8 Days	CHF 2600
Biomedicines biomedicines	3.9	5.2	2013	14.6 Days	CHF 2600

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Published Papers (4 papers)

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5 pages, 203 KiB  

Open AccessEditorial

Microangiopathy in Rheumatic Diseases

by Sevdalina Nikolova Lambova

Life 2023, 13(2), 491; https://doi.org/10.3390/life13020491 - 10 Feb 2023

Cited by 1 | Viewed by 1630

Abstract

Capillaries are part of the microcirculation, which consists of arterioles, capillaries, and venules and are the connecting link between the arterial and venous blood circulation [...] Full article

(This article belongs to the Topic Microangiopathy in Rheumatic Diseases)

13 pages, 292 KiB  

Open AccessReview

Nailfold Videocapillaroscopy for the Evaluation of Peripheral Microangiopathy in Rheumatoid Arthritis

by Panagiota Anyfanti, Elena Angeloudi, Athanasia Dara, Alexandra Arvanitaki, Eleni Bekiari, George D. Kitas and Theodoros Dimitroulas

Life 2022, 12(8), 1167; https://doi.org/10.3390/life12081167 - 31 Jul 2022

Cited by 18 | Viewed by 2486

Abstract

Rheumatoid arthritis (RA) is a chronic and refractory autoimmune joint disease that affects multiple organs. Several methods have been applied for the study of microvascular endothelial dysfunction, which is considered an important component of vascular disease in RA. Implementation of nailfold videocapillaroscopy (NVC) [...] Read more.

Rheumatoid arthritis (RA) is a chronic and refractory autoimmune joint disease that affects multiple organs. Several methods have been applied for the study of microvascular endothelial dysfunction, which is considered an important component of vascular disease in RA. Implementation of nailfold videocapillaroscopy (NVC) represents a viable choice, as the skin is an easily accessible window for the non-invasive, real-time assessment of subtle microcirculation abnormalities. Although NVC is routinely used in the rheumatology field, especially for the diagnostic workout of Raynaud’s phenomenon, accumulating evidence suggests a role in the evaluation of systemic vasculopathy associated with autoimmune rheumatic disorders. The current paper aims to provide an overview of NVC as a valuable clinical aid for the assessment of peripheral microcirculation in RA. Previous studies characterizing the capillaroscopic pattern in RA are summarized, along with associations with disease-related characteristics. Most available reports have mainly focused on the descriptions of non-specific morphological alterations that may reflect endothelial injury over the course of the disease. Still, the exact pattern of structural and functional capillaroscopic alterations and their clinical significance in RA remains a subject of ongoing research. Full article

(This article belongs to the Topic Microangiopathy in Rheumatic Diseases)

11 pages, 1476 KiB  

Open AccessArticle

Capillaroscopy and Immunological Profile in Systemic Sclerosis

by Sevdalina Nikolova Lambova, Ekaterina Krasimirova Kurteva, Sanie Syuleymanova Dzhambazova, Georgi Hristov Vasilev, Dobroslav Stanimirov Kyurkchiev and Mariela Gencheva Geneva-Popova

Life 2022, 12(4), 498; https://doi.org/10.3390/life12040498 - 29 Mar 2022

Cited by 8 | Viewed by 3864

Abstract

Introduction: Data on the associations between capillaroscopic changes and diagnostic systemic-sclerosis (SSc)-related antibodies are scarce. Presence of such correlation would improve current knowledge about the disease’s pathogenesis by revealing the mechanisms of microangiopathy. The microvascular pathology of SSc is a hallmark of the [...] Read more.

Introduction: Data on the associations between capillaroscopic changes and diagnostic systemic-sclerosis (SSc)-related antibodies are scarce. Presence of such correlation would improve current knowledge about the disease’s pathogenesis by revealing the mechanisms of microangiopathy. The microvascular pathology of SSc is a hallmark of the disease, and immunological abnormalities probably contribute to its development. Patients and methods: 19 patients with definite diagnosis of SSc were included in the current pilot study; 16 had limited and 3 had diffuse cutaneous involvement; their mean age was 51.56 ± 15.07 years. All patients exhibited symptoms of Raynaud’s phenomenon of the fingers. A “scleroderma” type capillaroscopic pattern was classified according to the staging suggested by Cutolo et al. (2000): “early”, “active” or ”late” phase. In the presence of different degrees of capillaroscopic changes in different fingers, the most-advanced microvascular pathology was chosen for classification. In cases without capillaroscopic features of microangiopathy, the findings were categorized as normal or nonspecific (dilated, tortuous capillaries, and/or hemorrhages). Indirect immunofluorescence on HEp-2 cells was performed as the gold-standard screening method for the detection of antinuclear autoantibodies (ANA), and determination of the immunofluorescent staining pattern (anti-cell pattern) was in accordance with the International Consensus on ANA Patterns. Scleroderma-associated autoantibodies in the patients’ serum were assessed using line immunoblot assay for detection of autoantibodies to 13 scleroderma-associated autoantigens: Scl-70, CENP A, CENP B, RP11/RNAP-III, RP155/RNAP-III, fibrillarin, NOR-90, Th/To, PM-Scl100, PM-Scl75, Ku, PDGFR, and Ro-52. Results: In 73.7% (n = 14) of the examined patients, “scleroderma” type capillaroscopic changes were found, and in 26.3% (n = 5), capillaroscopic features of microangiopathy were absent (nonspecific changes, n = 3; normal findings, n = 2). In SSc patients with positive anti-Scl-70 (n = 7) antibodies, significantly lower mean capillary density was observed along with a higher frequency of “active” and “late” phase capillaroscopic changes as compared to the anti-Scl-70-negative patients (p < 0.05). Anti-RNAP III–155 positive patients (n = 4) had significantly higher mean capillary density than anti-RNAP III–155 negative patients (n = 15). In three of the anti-RNAP III–155-positive cases, capillaroscopic features of microangiopathy were not detected, and in one case there was an “early” phase “scleroderma” pattern. Conclusion: In the current pilot study, the association between more advanced capillaroscopic changes and the presence of anti-Scl-70 autoantibodies was confirmed. As a novel observation, positive anti-RNAP III–155 antibodies were found in SSc patients with or without early microangiopathy. The question of associations between microvascular changes in SSc and other SSc-related autoantibodies requires further research. Full article

(This article belongs to the Topic Microangiopathy in Rheumatic Diseases)

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10 pages, 1484 KiB  

Open AccessArticle

Microangiopathy in Inflammatory Diseases—Strategies in Surgery of the Lower Extremity

by Christoph Biehl, Lotta Biehl, Ingo Helmut Tarner, Ulf Müller-Ladner, Christian Heiss and Martin Heinrich

Life 2022, 12(2), 200; https://doi.org/10.3390/life12020200 - 28 Jan 2022

Cited by 1 | Viewed by 2123

Abstract

Background: Patients with an inflammatory disease frequently develop chronic angiopathy of the capillaries. Due to this pathology, there is an increased rate of complications in lower extremity surgical procedures. It is not uncommon for microangiopathic wound healing disorders to cause deep infections and [...] Read more.

Background: Patients with an inflammatory disease frequently develop chronic angiopathy of the capillaries. Due to this pathology, there is an increased rate of complications in lower extremity surgical procedures. It is not uncommon for microangiopathic wound healing disorders to cause deep infections and fistulas, which lead to prolonged courses and hospitalizations. In addition, adhesions and ossifications of the contractile elements occur regularly. This sometimes results in serious limitations of the mobility of the patients. The study aims to present the results of a combination of vacuum and physical therapy. Patient and methods: A retrospective study of six patients with systemic sclerosis undergoing joint-related procedures of the lower extremity between 2015 and 2020 was performed. In addition to characterization of the patients and therapy, special attention was paid to cutaneous wound healing, affection of the fascia and displacement layers, and sclerosis of the muscle and tendon insertion. Results: The characterized structures (skin, tendon, fascia) show pathological changes at the microangiopathic level, which are associated with delayed healing and less physical capacity. Early suture removal regularly results in secondary scar dehiscence. With a stage-adapted vacuum therapy with sanitation of the deep structures and later on a dermal vacuum system, healing with simultaneous mobilization of the patients could be achieved in our patient cohort. Conclusion: In the case of necessary interventions on the lower extremity, such as trauma surgery, additional decongestive measures in the sense of regular and sustained lymphatic therapy and adapted physiotherapy are indispensable. Full article

(This article belongs to the Topic Microangiopathy in Rheumatic Diseases)

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