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Plastic and Reconstructive Surgery.... Jun 2024Symptomatic neuroma represents a debilitating complication after major limb amputation. The regenerative peripheral nerve interface (RPNI) has emerged as a reproducible...
Symptomatic neuroma represents a debilitating complication after major limb amputation. The regenerative peripheral nerve interface (RPNI) has emerged as a reproducible and practical surgery aimed at mitigating the formation of painful neuroma. Although previous animal studies revealed axonal sprouting, elongation, and synaptogenesis of proximal nerve stump within the muscle graft in RPNI, there is a lack of reports confirming these physiological reactions at the histopathological level in human samples. This report presents a case of below-knee amputation with RPNI due to foot gangrene resulting from polyarteritis nodosa. Subsequently, an above-knee amputation was necessitated due to the exacerbation of polyarteritis nodosa, providing the opportunity for histopathological examination of the RPNI site. The examination revealed sprouting, elongation, and existence of neuromuscular junction of the tibial nerve within the grafted muscle. To the best of our knowledge, this is the first report demonstrating axonal sprouting, elongation, and possibility of synaptogenesis of the nerve stump within the grafted muscle in a human sample.
PubMed: 38855139
DOI: 10.1097/GOX.0000000000005878 -
Clinical Case Reports Jun 2024This pediatric case report underscores the importance of maintaining a high clinical suspicion for polyarteritis nodosa (PAN) in patients presenting with atypical...
KEY CLINICAL MESSAGE
This pediatric case report underscores the importance of maintaining a high clinical suspicion for polyarteritis nodosa (PAN) in patients presenting with atypical features, such as migratory arthritis and subcutaneous nodules. Importantly, it highlights the focus on the potential relationship between streptococcal infection and cutaneous PAN. Early recognition and prompt, aggressive treatment is critical, as PAN can be a life-threatening condition if left unmanaged. This case emphasizes the need for a multidisciplinary approach to effectively identify and manage this rare vasculitis disorder in the pediatric population.
ABSTRACT
Polyarteritis nodosa (PAN) is a rare and life-threatening vasculitis with diverse clinical presentations, posing a diagnostic challenge. Early recognition and prompt intervention are crucial to prevent organ damage. We present the case of an 8-year-old boy who exhibited atypical symptoms including migratory arthritis, myalgia, digital discoloration and ischemic changes, and subcutaneous nodules. Initial concerns for septic arthritis were ruled out. A comprehensive evaluation revealed elevated inflammatory markers and a confirmatory skin biopsy demonstrating active leukocytoclastic vasculitis, are highly suggestive of a diagnosis of PAN. Notably, elevated ASO titers suggested a possible concurrent streptococcal infection. The aggressive treatment approach with high-dose aspirin, steroids, methotrexate, and tocilizumab is justified given the severity of the patient's symptoms and the nature of the disease process. This case underscores the importance of considering PAN in the differential diagnosis for children presenting with atypical features. Early diagnosis and prompt intervention, including addressing potential infectious triggers, are crucial for optimal outcomes in pediatric PAN.
PubMed: 38827940
DOI: 10.1002/ccr3.9038 -
Reumatologia Clinica May 2024Infections in patients with systemic vasculitis represent one of the main causes of mortality. Corticosteroid use, immunosuppressive therapy, age, associated organic...
UNLABELLED
Infections in patients with systemic vasculitis represent one of the main causes of mortality. Corticosteroid use, immunosuppressive therapy, age, associated organic involvement and dialysis dependence are risk factors of infection.
OBJECTIVES
To determine the prevalence of severe infection and associated factors in patients diagnosed with ANCA-associated vasculitis (AAV) and Polyarteritis Nodosa (PAN).
METHODS
retrospective study was conduced in a single rheumatology center (2000-2018). We included patients diagnosed with AAV (Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Microscopic Polyangiitis (PAM) and Polyarteritis nodosa (PAN). Serious infectious events requiring hospitalisation or prolonged antibiotic/antiviral treatment, recurrent infection of Herpes Zoster Virus or opportunistic infections were evaluated. Sites of infection, isolated microorganisms and mortality related were analyzed.
RESULTS
105 patients were analyzed, follow-up time median 18 m, 58.7% were women and median age was 52 years. Types of vasculitis: 41.9% PAM, 16.2% EPGA, 40% GPA, 1.9% PAN. Constitutional, pulmonary, renal and otorhinolaryngology manifestations were the most frequent.
PREVALENCE OF INFECTION
34.2%, with a median of 3 months from diagnosis of vasculitis to the infectious event. Low respiratory tract (42.8%), sepsis (31.4%), and urinary tract (14.3%) were the most common sites of infections. Bacterial aetiology was the most prevalent (67.7%). Mortality at the first event was 14.3% and a 72.2% of patients were in the induction phase of treatment. Infectious events were significantly associated with age > 65 years (p = 0.030), presence of lung (p = 0.016) and renal involvement (p = 0.001), BVASv3 > 15, mortality (p = 0.0002).
CONCLUSIONS
The prevalence of infection was 34.2%. Lower airway infections, septicemia and urinary tract infections were the most prevalent. Infections were associated with renal and pulmonary involvement, age older than 65 years and score BVAS > 15. Severe infections were associated with mortality, especially in elderly patients.
Topics: Humans; Female; Male; Middle Aged; Retrospective Studies; Adult; Aged; Prevalence; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Polyarteritis Nodosa; Risk Factors; Infections; Opportunistic Infections
PubMed: 38821740
DOI: 10.1016/j.reumae.2024.05.004 -
Polish Archives of Internal Medicine May 2024
PubMed: 38809179
DOI: 10.20452/pamw.16759 -
Cureus Apr 2024Acute cutaneous necrosis is a rare presentation of polyarteritis nodosa (PAN). In this study, we report a presentation with symmetrical cutaneous necrosis of the lower...
Acute cutaneous necrosis is a rare presentation of polyarteritis nodosa (PAN). In this study, we report a presentation with symmetrical cutaneous necrosis of the lower limbs, which ascended upward at a rapid rate. A 47-year-old man presented with a fever of one day and pain in the feet for six days. He had no history of claudication. Upon examination, he was febrile, and subtle bluish discoloration was observed on the sole of his foot. There was a bilateral stocking-type paresthesia up to the ankle joint. His blood pressure on admission was 210/120 mmHg. Eight hours later, the pain subsided, but a left-sided foot drop was noted along with the paresthesia extending up both feet to approximately 10 cm above the medial malleolus. The feet turned black, and dark discoloration spread rapidly upward over the next 16 hours, and the skin became necrosed. A clinical diagnosis of vasculitis was established, and the patient received IV methylprednisolone at a daily dosage of 1 g for three days, effectively stopping the advancement of necrosis. This was followed by treatment with IV cyclophosphamide. A conclusive diagnosis of PAN was made, and the patient underwent wound debridement. After three months of physiotherapy, a successful skin graft was performed. Prompt identification of the underlying etiology is crucial to prevent the advancement of necrosis and save the limbs. When vasculitis is suspected, ruling out infectious causes is essential before starting early immunosuppressive treatment.
PubMed: 38770516
DOI: 10.7759/cureus.58649 -
Frontiers in Immunology 2024Eosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic... (Review)
Review Observational Study
INTRODUCTION
Eosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic vasculitis and arterial or venous thrombosis. Here we aim to report on the clinical features and treatment outcomes of patients with unexplained ophthalmic vascular manifestations and eosinophilia.
METHODS
We conducted a retrospective, multicenter, observational study and a literature review of patients with eosinophilia (≥0.5 x10/L) and concomitant ophthalmic vascular manifestations independent of the underlying eosinophilic disease but with no alternative cause for ophthalmic manifestations.
RESULTS
Fifty-seven patients were included (20 from the observational study and 37 from the literature review). Ophthalmic vascular features were the initial manifestation of eosinophil-related disease in 34 (59%) patients and consisted of 29 central retinal artery occlusions, six branch retinal artery occlusions, five central retinal vein occlusions, two branch retinal vein occlusions, seven retinal vasculitides, two retinal vasospasms, 12 Purtscher's retinopathies, 13 anterior ischemic optic neuropathies and two posterior ischemic optic neuropathies. The median [IQR] absolute eosinophil count at onset of ophthalmic vascular manifestations was 3.5 [1.7-7.8] x10/L. Underlying eosinophil-related diseases included eosinophilic granulomatosis with polyangiitis (n=32), clonal hypereosinophilic syndrome (HES) (n=1), idiopathic HES (n=13), lymphocytic HES (n=2), adverse drug reactions (n=3), parasitosis (n=2), polyarteritis nodosa (n=1), IgG4-related disease (n=1), eosinophilic fasciitis (n=1) and primary sclerosing cholangitis (n=1). Other extra-ophthalmologic arterial or venous thromboses related to eosinophilia were reported in four (7%) and nine (16%) patients, respectively. Visual prognosis was poor: only eight (10%) patients achieved full recovery of ophthalmologic symptoms. After a median follow-up of 10.5 [1-18] months, one patient (3%) had a recurrence of an ophthalmic vascular manifestation, and three patients (10%) had a recurrence of other vascular symptoms (deep vein thrombosis in two and pulmonary embolism in one patient). At the time of recurrence, absolute eosinophil counts were above 0.5 x10/L in all cases (n=4).
DISCUSSION
This study broadens the spectrum of vascular manifestations associated with hypereosinophilia by adding ophthalmic vascular manifestations. In patients with ophthalmological vascular manifestations and hypereosinophilia, aggressive treatment of the underlying pathology (and normalization of blood count) should be implemented.
Topics: Humans; Male; Middle Aged; Female; Retrospective Studies; Eosinophilia; Eosinophils; Aged; Adult
PubMed: 38720897
DOI: 10.3389/fimmu.2024.1379611 -
The Pan African Medical Journal 2024An arterial aneurysm is a localized weakening of the artery wall that results in pathological dilatation. All intra-abdominal artery aneurysms are labeled as visceral...
An arterial aneurysm is a localized weakening of the artery wall that results in pathological dilatation. All intra-abdominal artery aneurysms are labeled as visceral artery aneurysms (VAA), apart from the aorto-iliac artery aneurysms. VAA´s are rare, gastroduodenal artery aneurysms (GDAA), constituting 1.5% of visceral artery aneurysms. A woman in her early 80s´ presented with chronic epigastric pain, weight loss, and nausea. Conservative management was unsuccessful. Imaging revealed a GDAA, prompting endovascular coil embolization. Subsequent evaluation confirmed Polyarteritis Nodosa (PAN), treated with rituximab. The report underscores the diagnostic challenges, emphasizing the need for a multidisciplinary approach using imaging and angiography. GDAA's potential life-threatening rupture necessitates prompt intervention, as illustrated in this case. The rare association with PAN, although infrequent, underscores the importance of considering underlying etiologies in multiple visceral aneurysms. Early diagnosis and intervention are pivotal for this uncommon yet potentially lethal condition.
Topics: Humans; Female; Abdominal Pain; Embolization, Therapeutic; Aneurysm; Aged, 80 and over; Polyarteritis Nodosa; Rituximab; Duodenum; Angiography; Gastric Artery
PubMed: 38708132
DOI: 10.11604/pamj.2024.47.77.42605 -
Diagnostics (Basel, Switzerland) Apr 2024Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as... (Review)
Review
Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as such, diagnosis and subsequent management are challenging. These entities are typically classified by the size of vessel involved, including large-vessel vasculitis (giant cell arteritis, Takayasu's arteritis, and clinically isolated aortitis), medium-vessel vasculitis (including polyarteritis nodosa and Kawasaki disease), and small-vessel vasculitis (granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis). There are also other systemic vasculitides that do not fit in to these categories, such as Behcet's disease, Cogan syndrome, and IgG4-related disease. Advances in medical imaging modalities have revolutionized the approach to diagnosis of these diseases. Specifically, color Doppler ultrasound, computed tomography and angiography, magnetic resonance imaging, positron emission tomography, or invasive catheterization as indicated have become fundamental in the work up of any patient with suspected systemic or localized vasculitis. This review presents the key diagnostic imaging modalities and their clinical utility in the evaluation of systemic vasculitis.
PubMed: 38667483
DOI: 10.3390/diagnostics14080838 -
Journal of Dermatological Science May 2024Photoacoustic microscopy is expected to have clinical applications as a noninvasive and three-dimensional (3D) method of observing intradermal structures.
Visualization of intradermal blood vessel structures by dual-wavelength photoacoustic microscopy and characterization of three-dimensional construction of livedo-racemosa in cutaneous polyarteritis nodosa.
BACKGROUND
Photoacoustic microscopy is expected to have clinical applications as a noninvasive and three-dimensional (3D) method of observing intradermal structures.
OBJECTIVE
Investigate the applicability of a photoacoustic microscope equipped with two types of pulsed lasers that can simultaneously recognize hemoglobin and melanin.
METHODS
16 skin lesions including erythema, pigmented lesions, vitiligo and purpura, were analyzed to visualize 3D structure of melanin granule distribution and dermal blood vessels. 13 cases of livedo racemosa in cutaneous polyarteritis nodosa (cPN) were further analyzed to visualize the 3D structure of dermal blood vessels in detail. Vascular structure was also analyzed in the biopsy specimens obtained from tender indurated erythema of cPN by CD34 immunostaining.
RESULTS
Hemoglobin-recognition signal clearly visualized the 3D structure of dermal blood vessels and melanin-recognition signal was consistently reduced in vitiligo. In livedo racemosa, the hemoglobin-recognition signal revealed a relatively thick and large reticular structure in the deeper layers that became denser and finer toward the upper layers. The numerical analysis revealed that the number of dermal blood vessels was 1.29-fold higher (p<0.05) in the deeper region of the lesion than that of normal skin. The CD34 immunohistochemical analysis in tender indurated erythema revealed an increased number of dermal vessels compared with normal skin in 88.9% (8/9) of the cases, suggesting that vascular network remodeling had occurred in cPN.
CONCLUSION
The photoacoustic system has an advantage in noninvasively detecting dermal blood vessel structures that are difficult to recognize by two-dimensional histopathology specimen examination and is worth evaluating in various skin diseases.
Topics: Humans; Photoacoustic Techniques; Male; Middle Aged; Female; Melanins; Adult; Imaging, Three-Dimensional; Polyarteritis Nodosa; Skin; Aged; Blood Vessels; Hemoglobins; Biopsy; Young Adult; Microscopy; Livedo Reticularis; Antigens, CD34
PubMed: 38644095
DOI: 10.1016/j.jdermsci.2024.03.010 -
International Journal of Molecular... Mar 2024Chronic primary systemic vasculitis (PSV) comprises a group of heterogeneous diseases that are broadly classified by affected blood vessel size, clinical traits and the...
Chronic primary systemic vasculitis (PSV) comprises a group of heterogeneous diseases that are broadly classified by affected blood vessel size, clinical traits and the presence (or absence) of anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3 (PR3) and myeloperoxidase (MPO). In small vessel vasculitis (SVV), ANCA are not present in all patients, and they are rarely detected in patients with vasculitis involving medium (MVV) and large (LVV) blood vessels. Some studies have demonstrated that lysosome-associated membrane protein-2 (LAMP-2/CD107b) is a target of ANCA in SVV, but its presence and prognostic value in childhood MVV and LVV is not known. This study utilized retrospective sera and clinical data obtained from 90 children and adolescents with chronic PSV affecting small (SVV, n = 53), medium (MVV, n = 16), and large (LVV, n = 21) blood vessels. LAMP-2-ANCA were measured in time-of-diagnosis sera using a custom electrochemiluminescence assay. The threshold for seropositivity was established in a comparator cohort of patients with systemic autoinflammatory disease. The proportion of LAMP-2-ANCA-seropositive individuals and sera concentrations of LAMP-2-ANCA were assessed for associations with overall and organ-specific disease activity at diagnosis and one-year follow up. This study demonstrated a greater time-of-diagnosis prevalence and sera concentration of LAMP-2-ANCA in MVV (52.9% seropositive) and LVV (76.2%) compared to SVV (45.3%). Further, LAMP-2-ANCA-seropositive individuals had significantly lower overall, but not organ-specific, disease activity at diagnosis. This did not, however, result in a greater reduction in disease activity or the likelihood of achieving inactive disease one-year after diagnosis. The results of this study demonstrate particularly high prevalence and concentration of LAMP-2-ANCA in chronic PSV that affects large blood vessels and is seronegative for traditional ANCA. Our findings invite reconsideration of roles for autoantigens other than MPO and PR3 in pediatric vasculitis, particularly in medium- and large-sized blood vessels.
Topics: Adolescent; Child; Humans; Antibodies, Antineutrophil Cytoplasmic; Autoantigens; Myeloblastin; Retrospective Studies; Systemic Vasculitis
PubMed: 38612581
DOI: 10.3390/ijms25073771