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Cureus Sep 2023Polyarteritis nodosa (PAN) is a heterogeneous disease characterized by constitutional symptoms, multi-organ dysfunction, and a subacute to chronic mode of onset. We...
Polyarteritis nodosa (PAN) is a heterogeneous disease characterized by constitutional symptoms, multi-organ dysfunction, and a subacute to chronic mode of onset. We describe a rarely encountered manifestation of PAN in a previously healthy 64-year-old woman who developed acute intra-abdominal bleeding due to multi-vessel, vasculitis-induced retroperitoneal aneurysmal ruptures, followed by the spontaneous cessation of abdominal pain and intra-abdominal bleeding within 24 hours after hospitalization, in the absence of immunosuppressive treatment. Aneurysms regressed and eventually normalized coincident with a moderate-term course of oral corticosteroids and six months of monthly intravenous cyclophosphamide. Our report reveals that patients with life-threatening PAN may present acutely with unexpected historical, physical examination and laboratory features not commensurate with the gravity of the clinical findings. We also highlight that long-term survival lasting greater than 11 years can occur when the correct diagnosis and appropriate treatment are introduced early in the course of the illness.
PubMed: 37868509
DOI: 10.7759/cureus.45718 -
Current Rheumatology Reviews 2024Malignancy-associated vasculitis usually presents in the form of polyarteritis nodosa or leukocytoclastic vasculitis. However, ANCA vasculitis associated with malignancy...
BACKGROUND
Malignancy-associated vasculitis usually presents in the form of polyarteritis nodosa or leukocytoclastic vasculitis. However, ANCA vasculitis associated with malignancy is rare. Here, we present a case of MPO+ ANCA vasculitis with pauci-immune GN associated with invasive ductal carcinoma of the breast.
CASE PRESENTATION
A 66-year-old female with a history of rheumatoid arthritis, Hashimoto's thyroiditis, and psoriasis presented with multiple joint pain, body aches, petechial rash, paresthesia and numbness, and deranged renal function a month after diagnosis of localized left breast invasive ductal carcinoma. Renal biopsy showed crescentic pauci-immune glomerulonephritis, and serology was positive for Perinuclear Antineutrophil Cytoplasmic Antibody (P-ANCA) and myeloperoxidase (MPO). The disease course was complicated by diverticulitis with peritonitis and intraperitoneal abscess collection, which required laparoscopic peritoneal lavage and additional interventional radiology-guided drainage of the abscess. We treated the patient successfully with steroids, rituximab, and mastectomy for left breast malignant lesions, resulting in the resolution of symptoms, normalization of inflammatory markers, and ANCA seroconversion.
CONCLUSION
Treating ANCA-associated Vasculitis (AAV) in surgical emergencies like bowel perforation can be challenging. Individualized treatment strategy tailored to patients' acute needs is crucial. In this case, we considered malignancy-associated vasculitis and pursued treatment that fit the patient's clinical situation in a multidisciplinary approach.
Topics: Female; Humans; Aged; Antibodies, Antineutrophil Cytoplasmic; Glomerulonephritis; Abscess; Breast Neoplasms; Mastectomy; Vasculitis; Peroxidase; Carcinoma, Ductal
PubMed: 37855288
DOI: 10.2174/0115733971246438230924163114 -
QJM : Monthly Journal of the... Feb 2024
Topics: Humans; Coronary Aneurysm; Polyarteritis Nodosa; Twins, Monozygotic
PubMed: 37843445
DOI: 10.1093/qjmed/hcad239 -
Cureus Oct 2023Polyarteritis nodosa (PAN) is a rare systemic vasculitis characterised by necrotising inflammation of medium-sized arteries. PAN can affect patients of any age, gender,...
Polyarteritis nodosa (PAN) is a rare systemic vasculitis characterised by necrotising inflammation of medium-sized arteries. PAN can affect patients of any age, gender, or ethnic background. Its highest incidence is in the fifth-sixth decade of life, with a slight male-to-female predilection. PAN can be idiopathic or secondary to a multitude of systemic conditions, such as infection, haematological malignancy, or autoinflammatory disorders. PAN has a broad spectrum of possible clinical manifestations the most common being constitutional symptoms, such as fever and myalgia. While cardiac involvement is well-described and is a common cause of mortality, it is exceedingly uncommon as the initial presentation. Below, we describe a case of a female in her 60s who presented with pericarditis as the first manifestation of PAN.
PubMed: 37822689
DOI: 10.7759/cureus.46717 -
Rheumatic Diseases Clinics of North... Nov 2023Deficiency of adenosine deaminase 2 (DADA2) is a monogenic vasculitis syndrome caused by biallelic mutations in the adenosine deaminase 2 gene. The diagnosis of DADA2 is... (Review)
Review
Deficiency of adenosine deaminase 2 (DADA2) is a monogenic vasculitis syndrome caused by biallelic mutations in the adenosine deaminase 2 gene. The diagnosis of DADA2 is confirmed by decreased enzymatic activity of ADA2 and genetic testing. Symptoms range from cutaneous vasculitis and polyarteritis nodosa-like lesions to stroke. The vasculopathy of DADA2 can affect many organ systems, including the gastrointestinal and renal systems. Hematologic manifestations occur early with hypogammaglobulinemia, lymphopenia, pure red cell aplasia, or pancytopenia. Treatment can be challenging. Tumor necrosis factor inhibitors are helpful to control inflammatory symptoms. Hematopoietic stem cell transplant may be needed to treat refractory cytopenias, vasculopathy, or immunodeficiency.
Topics: Humans; Adenosine Deaminase; Intercellular Signaling Peptides and Proteins; Polyarteritis Nodosa; Vasculitis; Mutation
PubMed: 37821195
DOI: 10.1016/j.rdc.2023.06.004 -
Oxford Medical Case Reports Sep 2023Different types of vasculitis have been reported after various vaccine administrations. Recently, the coronavirus disease 2019 (COVID-19) vaccine was one of the most...
Different types of vasculitis have been reported after various vaccine administrations. Recently, the coronavirus disease 2019 (COVID-19) vaccine was one of the most common vaccine-induced vasculitis. Herein, we describe a 56-year-old male patient with chronic hepatitis B who presented with abdominal pain for 2 days, which was associated with vomiting and bloody diarrhea. He had a history of petechial rash for 25 days, multiple joint pain and lower limb weakness after the second dose of the COVID-19 vaccine. A skin biopsy showed medium-sized vessel vasculitis. Polyarteritis nodosa (PAN) was diagnosed depending on the American College of Rheumatology criteria. He was treated with steroids, plasmapheresis and antiviral medication with a good prognosis. In patients with a past medical history of chronic hepatitis B, the covid vaccine may be associated with an increased risk of developing a PAN, so clinicians should suspect the occurrence of this disease after COVID-19 vaccination.
PubMed: 37771685
DOI: 10.1093/omcr/omad092 -
Cureus Aug 2023Polyarteritis nodosa (PAN) is a rare necrotizing vasculitis that affects medium-sized arteries. The association of hepatitis B virus (HBV)and HIV with PAN is well...
Polyarteritis nodosa (PAN) is a rare necrotizing vasculitis that affects medium-sized arteries. The association of hepatitis B virus (HBV)and HIV with PAN is well documented. Although there are documented cases of PAN in patients with hepatitis C virus (HCV) infection, the connection between PAN and HCV is not well established. We report a case of PAN in a patient with HCV infection who failed treatment with interferon.
PubMed: 37753027
DOI: 10.7759/cureus.44129 -
Archives of Cardiovascular Diseases Oct 2023Aortic valve stenosis is associated with age, rheumatic fever and bicuspid aortic valve, but its association with other co-morbidities, such as inflammatory disease and...
BACKGROUND
Aortic valve stenosis is associated with age, rheumatic fever and bicuspid aortic valve, but its association with other co-morbidities, such as inflammatory disease and race/ethnicity, is less known.
AIM
To investigate any association between aortic stenosis and many co-morbidities.
METHODS
We used the large Nationwide Inpatient Sample database to evaluate any association between aortic stenosis and risk factors. We performed univariate and multivariable analyses, adjusting for co-morbid conditions.
RESULTS
Data were extracted from the first available database that used the International Classification of Diseases, Tenth Revision codes specifically coding for aortic stenosis alone, spanning from 2016 to 2020 (n=112,982,565). A total of 2,322,649 patients had aortic stenosis; the remaining 110,659,916 served as controls. We found a strong and independent significant association between aortic stenosis and coronary artery disease (odds ratio [OR]: 2.11, 95% confidence interval [CI]: 2.09-2.13), smoking (OR: 1.08, 95% CI: 1.07-1.08), diabetes mellitus (OR: 1.15, 95% CI: 1.14-1.16), hypertension (OR: 1.41, 95% CI: 1.4-1.42), hyperlipidaemia (OR: 1.31, 95% CI: 1.3-1.32), renal disease (OR: 1.3, 95% CI: 1.29-1.31), chronic obstructive pulmonary disease (OR: 1.05, 95% CI: 1.04-1.05), obesity (OR: 1.3, 95% CI: 1.29-1.32), white race/ethnicity (OR: 1.47, 95% CI: 1.42-1.52), rheumatoid arthritis (OR: 1.13, 95% CI: 1.11-1.15), scleroderma (OR: 1.93, 95% CI: 1.79-2.09), systemic connective tissue disease (OR: 1.24, 95% CI: 1.2-1.27), polyarteritis nodosa (OR: 1.5, CI: 1.24-1.81) and Raynaud's syndrome (OR: 1.16, 95% CI: 1.09-1.24) (all P<0.001), in addition to known factors, such as age, male sex and bicuspid aortic valve.
CONCLUSION
Using a very large database, we found many new associations with aortic valve stenosis, including race/ethnicity, renal disease, several inflammatory diseases, chronic obstructive pulmonary disease and obesity, in addition to many other known cardiovascular risk factors.
PubMed: 37749002
DOI: 10.1016/j.acvd.2023.07.008 -
Explore (New York, N.Y.) 2024Cutaneous polyarteritis nodosa (cPAN) is a systemic disease that is limited to the skin. cPAN usually presents with cutaneous reticular cyanotic, erythematous and...
BACKGROUND
Cutaneous polyarteritis nodosa (cPAN) is a systemic disease that is limited to the skin. cPAN usually presents with cutaneous reticular cyanotic, erythematous and palpable nodules, and cutaneous ulcers.Research has indicated that the use of hormones and immunosuppressive drugs can delay ulcer healing and associated neuropathy, and also elevate the risk of disease recurrence upon their reduction or withdrawal. Therefore, it is a necessary to find a safe and effective approach that minimize hormone side effects in ulcer treatment.
CASE PRESENTATION
The patient, a 48-year-old female of Han Chinese ethnicity, has suffered from recurrent erythema nodosum on both lower limbs for 8 years. The condition was aggravated by skin breakdown over the last 3 months. Despite multiple treatments, the patient's condition did not improve significantly, leading to the exploration of a combined approach of traditional Chinese and Western medicine. Following six months of combined traditional Chinese and Western medicine treatment, t the patient's newborn erythema and ulcers on both lower limbs did not reappear, and the ulcers gradually decreased in size and the erythema disappeared. The patient took the TCM regularly until April 15, 2023, when the ulcers were completely healed. Three months after the patient stopped taking TCM, the ulcers had completely healed with no recurrence, as observed during the follow-up visit on July 14th, 2023.
CONCLUSION
Traditional Chinese Medicine Combined with Low-Dose Hormones May Effectively Treat Bilateral Lower Extremity Skin Ulcers Caused by Cutaneous Polyarteritis Nodosa.
Topics: Female; Infant, Newborn; Humans; Middle Aged; Polyarteritis Nodosa; Ulcer; Lower Extremity; Erythema; Hormones
PubMed: 37743154
DOI: 10.1016/j.explore.2023.08.018 -
Northern Clinics of Istanbul 2023The aim of this study is to evaluate the clinical, laboratory, and radiological findings and prognosis of patients with adenosine deaminase 2 deficiency (DADA2) and to...
OBJECTIVE
The aim of this study is to evaluate the clinical, laboratory, and radiological findings and prognosis of patients with adenosine deaminase 2 deficiency (DADA2) and to highlight the conditions that DADA2 should be considered in the differential diagnosis in patients with neurological findings.
METHODS
A case series of six DADA2 patients was presented in this retrospective, descriptive study. Clinical and laboratory data, treatment protocols, and prognosis of the patients were recorded. A diagnosis of DADA2 was established by ADA2 enzyme activity assay and/or ADA2 gene sequencing.
RESULTS
Six patients with DADA2 were included in the study. The median age at symptom onset was 6.5 years (range 3.5-13.5 years). The median time to diagnosis from the initial presentation was 9 (3-72) months. Consanguinity was present in the families of 4 cases. The skin, nervous system, and musculoskeletal system were the most commonly involved systems. Vasculitis mimicking polyarteritis nodosa (PAN) was the predominant phenotype (n=4) in our case series. Four patients with PAN-like features had neurological involvement. Ischemic strokes were found in 3 patients, cranial nerve palsy in 2 patients, and seizures in 2 patients. The CECR1 gene was analyzed in all patients. We analyzed plasma ADA2 enzyme activity only in one patient. Anti-tumor necrosis factor (TNF)-α therapy was initiated. Inflammation was suppressed and remission was achieved in all patients.
CONCLUSION
DADA2 should be considered in patients with PAN-like disease, a history of familial PAN/vasculitis, early-onset strokes/neurological involvement with systemic inflammation. Furthermore, anti-TNF-α therapy appears to be beneficial for the treatment of DADA2.
PubMed: 37719263
DOI: 10.14744/nci.2022.45380