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Nailfold capillaroscopy improvement after immunosupressive treatment. Fourth finger nailfold capillaroscopy performed ( A ) before immunosuppressive treatment showing megacapillaries (black arrow) and hemorrhages (black arrow tip). Fourth finger nailfold capillaroscopy repeated ( B ) one year after immunosuppressive treatment showing tortuosities (white arrow) and ramifications (white arrow tip).
Source publication
A 74-year-old woman with history of Raynaud phenomenon started 10 years ago, vitiligo and Graves-Basedow disease treated at age 53 years with radioactive iodine, presented to the Department of Internal Medicine of the Vall d'Hebron University Hospital in January 2014, with 4 months of progressive muscle weakness. She also had dyspnea on exertion an...
Contexts in source publication
Context 1
... At this point, we performed a nailfold capillaroscopy that showed widespread capillary hemorrhages and megacapillaries (Figure 1 and Supplementary Appendix S1). Once polymyositis diagnosis was established, the patient was started on monthly intravenous immune globulin, aza- thioprine and oral prednisone 1 mg/kg per day that was slowly tapered during 1 year to 5 mg daily. One year after the initial evaluation there was not periungeal erythema, power in all muscle groups was 5/5 and both pulmonary function tests and muscle enzymes were normal. A follow-up capillaroscopy showed a marked improvement of the micro- vascular involvement with resolution of the megacapillaries and the hemorrhages (Figure 1, Supplementary Appendix S1 and video). Nailfold capillaroscopy is a non-invasive method to assess skin microvasculature mainly used to discriminate ...
Context 2
... At this point, we performed a nailfold capillaroscopy that showed widespread capillary hemorrhages and megacapillaries (Figure 1 and Supplementary Appendix S1). Once polymyositis diagnosis was established, the patient was started on monthly intravenous immune globulin, aza- thioprine and oral prednisone 1 mg/kg per day that was slowly tapered during 1 year to 5 mg daily. One year after the initial evaluation there was not periungeal erythema, power in all muscle groups was 5/5 and both pulmonary function tests and muscle enzymes were normal. A follow-up capillaroscopy showed a marked improvement of the micro- vascular involvement with resolution of the megacapillaries and the hemorrhages (Figure 1, Supplementary Appendix S1 and video). Nailfold capillaroscopy is a non-invasive method to assess skin microvasculature mainly used to discriminate ...
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Introducción:
Entre un 15 y un 20% de los pacientes con fenómeno de Raynaud progresarán a una enfermedad autoinmune sistémica. La presencia de autoanticuerpos o alteraciones capilaroscópicas es fundamental para el diagnóstico temprano.
Objetivos:
Determinar las características de la videocapilaroscopia del lecho ungular y de los anticuerpos antinu...
The aim of this study was to analyze the relationship between the qualitative abnormalities on nailfold videocapillaroscopy (NVC), and the concentrations of selected biomarkers (sE-selectin, endothelin-1, high-sensitivity c-reactive protein (hsCRP)) and lipid metabolism parameters in children and adolescents with Raynaud’s phenomenon (RP). Raynaud’...
Background
Utilising fluorescence optical imaging (FOI), the distribution of an intravenously applied colouring agent indocyanine green (ICG) can be analysed with the potential to identify malperfusion by little to no tissue enhancement. Systemic sclerosis (SSc) is characterised by the presence of digital ulcers reflecting progressive vasculopathy....
Objectives: Raynaud’s phenomenon (RP) is characterized by intense vasospasm of the digital arteries that causes characteristic color changes in fingers. There are two main types of RP: Primary RP (PRP) and Secondary RP (SRP). PRP is a benign condition. Whilst SRP is associated with several connective tissue diseases (CTD), in particular systemic sc...
Citations
... Another observation made during this research project is the higher prevalence of elongated capillaries among patients with vitiligo. According to the author's knowledge, literature data on capillaroscopic images in patients with vitiligo mainly concern people suffering from comorbidities and do not indicate the occurrence of changes typical of vitiligo in capillaroscopy [37,38]. In addition, healthy people may have elongated vessels [12]. ...
Aims/Hypothesis
The study aimed to assess the usefulness of capillaroscopy and photoplethysmography in the search for early vascular anomalies in children with type 1 diabetes.
Methods
One hundred sixty children and adolescents aged 6–18, 125 patients with type 1 diabetes, and 35 healthy volunteers were enrolled in the study. We performed a detailed clinical evaluation, anthropometric measurements, nailfold capillaroscopy, and photoplethysmography.
Results
Patients with diabetes had more often abnormal morphology in capillaroscopy (68.60%, p = 0.019), enlarged capillaries (32.6%, p = 0.006), and more often more over five meandering capillaries (20.90%, p = 0.026) compared to healthy controls. Meandering capillaries correlated with higher parameters of nutritional status. In a photoplethysmography, patients with diagnosed neuropathy had a higher percentage of flow disturbance curves (p < 0.001) with a reduced frequency of normal curves (p = 0.050).
Conclusions
Capillaroscopic and photoplethysmographic examinations are non-invasive, painless, fast, and inexpensive. They are devoid of side effects, and there are no limitations in the frequency of their use and repetition. The usefulness of capillaroscopy and photoplethysmography in the study of microcirculation in diabetic patients indicates the vast application possibilities of these methods in clinical practice.
... Dynamics and significant improvement of capillaroscopic changes impacted by the ap-plied treatment can also be observed in other connective tissue systemic diseases, such as dermatomyositis or polyamositis. It was demonstrated that the immunosuppressive therapy significantly increases the density of capillaries and improves their distribution the group of patients with microangiopathy typical for systemic sclerosis [18,19]. ...
... Dynamikę i istotną poprawę zmian kapilaroskopowych pod wpływem stosowanego leczenia również można zaobserwować w innych chorobach układowych tkanki łącznej, takich jak zapalenie skórno-mięśniowe czy wielomięśniowe. Wykazano, iż w tej grupie chorych z mikroangiopatią typową dla twardziny leczenie immunosupresyjne istotnie zwiększa gęstość kapilar i poprawia ich rozmieszczenie [18,19]. ...
... Another important finding in the present study was the improvement and disappearance of NVC scleroderma spectrum abnormalities in IIM patients after 1 year of treatment with immunosuppressive drugs. A previous two case reports showed similar findings [41,42]. ...
Objective:
The aim of this study was to investigate the clinical and immunological significance of nailfold videocapillaroscopy (NVC) abnormalities in patients with idiopathic inflammatory myopathies (IIMs).
Methods:
Seventy consecutive Japanese patients with untreated IIMs, enrolled between April 2014 and August 2017, were prospectively studied. Clinical features, NVC findings, autoantibody profile by immunoprecipitation and ELISA, and histopathological findings of skin biopsies of DM rash were assessed at baseline and after 1-year of immunosuppressive therapy.
Results:
NVC abnormalities were found in 55.7% (39/70) of IIM patients, with significantly higher prevalence in DM (65.4%) compared with PM (27.8%) (P = 0.01). In subsets of patients classified by autoantibody specificities, the prevalence of NVC abnormalities was significantly higher in patients with anti-MDA5 (87.5%) and anti-transcriptional intermediary factor 1γ (88.9%) vs anti-aminoacyl-tRNA synthetase (26.9%, P < 0.001). Perivascular lymphocytic infiltration in the upper dermis of skin rash biopsy of DM was more severe in patients with NVC abnormalities (P < 0.05). Unexpectedly, NVC abnormalities disappeared in 75% of IIM patients after 1-year of immunosuppressive therapy in contrast to stable NVC changes seen in scleroderma patients.
Conclusion:
Nailfold microvascular abnormalities were common in DM patients, associated with anti-MDA5 and transcriptional intermediary factor 1γ antibodies, and perivascular inflammation in skin histology. NVC abnormalities in IIMs may become clinically useful markers for defining subsets of DM and understanding the pathogenesis of the clinical features seen in these patients.
Introduction:
Dermatomyositis (DM) is a rare inflammatory disease with diverse cutaneous and systemic manifestations, often associated with myositis-specific antibodies. Managing patients with refractory DM, or individuals presenting pecific complications, like calcinosis or rapidly progressive interstitial lung disease, presents unique challenges.
Areas covered:
This review explores current and promising treatment options for DM, drawing from clinical studies, case series, and case reports that consider the underlying disease pathophysiology.
Expert opinion:
Recent advancements have improved our understanding and management of DM. The discovery of distinct DM autoantibodies and their correlation with specific clinical phenotypes has transformed patient categorization and enhanced our knowledge of the pathogenesis of the disease. Intravenous immunoglobulin, a well-established treatment in dermatomyositis, has regained prominence and a large randomized clinical trial has reaffirmed its efficacy, confirming it as an effective therapeutic option in this group of patients. Identification of the type I interferon pathway as a key pathogenic mechanism in DM has opened up new avenues for more effective treatment strategies. Blocking the JAK/STAT pathway offers potential for improved management of refractory patients and prevention of highly morbid complications. These recent advancements have significantly impacted the management and care of dermatomyositis patients, enabling tailored approaches, targeted interventions, and improved outcomes for individuals affected by this complex condition.
The extramuscular features of the idiopathic inflammatory myopathies (IIM) include arthritis, Raynaud’s phenomenon and constitutional symptoms such as fatigue, fever and weight loss. These features may predate the onset of myositis symptoms. Arthritis most commonly manifests in patients with anti-Jo-1 autoantibodies and the anti-synthetase syndrome, as does Raynaud’s phenomenon. The arthritis seen in this subgroup of patients is typically non-erosive, but can be deforming. Constitutional symptoms are a common early symptom but also reoccur with disease flares. In this chapter, we aim to present the extramuscular features of myositis patients and find in which subgroups they frequently occur.
