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BMJ Case Reports Jun 2024A man in his 70s presented with a sudden onset stabbing back pain radiating to the chest and pre-syncopal symptoms. He underwent urgent investigations, including a CT...
A man in his 70s presented with a sudden onset stabbing back pain radiating to the chest and pre-syncopal symptoms. He underwent urgent investigations, including a CT angiogram aorta which did not reveal any abnormalities within the thorax, abdomen or pelvis and no cause of symptoms was identified. After being discharged, he re-presented 2 days later with syncopal episodes, abdominal pain and a significant drop in haemoglobin levels. This time, a CT mesenteric angiogram showed two hepatic artery pseudoaneurysms and a large haemoperitoneum. Following a hepatic artery embolisation, a workup showed that the likely cause of the pseudoaneurysms was a rare first presentation of polyarteritis nodosa. This case highlights the importance of considering the possibility of an aneurysmal rupture, especially when common causes of an acute abdomen have been excluded, and not relying on previous negative investigations to exclude pathology, as the outcomes can be detrimental.
Topics: Humans; Polyarteritis Nodosa; Aneurysm, False; Male; Hepatic Artery; Aged; Embolization, Therapeutic; Aneurysm, Ruptured; Computed Tomography Angiography; Rupture, Spontaneous; Hemoperitoneum; Abdominal Pain
PubMed: 38937262
DOI: 10.1136/bcr-2023-257411 -
BMJ Case Reports Jun 2024SummaryUlcerative colitis (UC), a chronic inflammatory bowel disease, can cause extraintestinal manifestations (EIMs) in approximately 40% of individuals. This case...
SummaryUlcerative colitis (UC), a chronic inflammatory bowel disease, can cause extraintestinal manifestations (EIMs) in approximately 40% of individuals. This case report discusses the diagnostic procedure of a woman in her 20s who initially had non-specific symptoms. The patient underwent a thorough evaluation, which initially pointed towards tuberculosis (TB) due to necrotic lymphadenopathy and granulomatous hepatitis. However, no microbiological evidence of TB was found, and her symptoms worsened despite antitubercular therapy. The patient developed painful nodular-ulcerative skin lesions consistent with cutaneous polyarteritis nodosa (cPAN) on biopsy. Eventually, a definitive diagnosis of UC was made, revealing the true nature of her multisystemic manifestations. Cutaneous vasculitis, including leucocytoclastic vasculitis and cPAN, is a rare EIM of UC, with only five reported cases in the literature. This case report highlights the clinical implications of EIMs and contributes to the expanding knowledge of rare EIMs such as cPAN and granulomatous hepatitis.
Topics: Humans; Polyarteritis Nodosa; Female; Colitis, Ulcerative; Hepatitis; Diagnosis, Differential; Granuloma; Adult; Antitubercular Agents
PubMed: 38926121
DOI: 10.1136/bcr-2023-257581 -
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 -
Rheumatology (Oxford, England) Jun 2024
PubMed: 38848474
DOI: 10.1093/rheumatology/keae054 -
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 -
BMJ Case Reports May 2024A girl in the early adolescent age group presented with multisystem manifestations in the form of periodic fever, recurrent abdominal pain, hypertension, seizure, skin...
A girl in the early adolescent age group presented with multisystem manifestations in the form of periodic fever, recurrent abdominal pain, hypertension, seizure, skin lesions over the chest and gangrene over the left ring and middle fingertips. Her condition had remained undiagnosed for 11 years. On evaluation, she had features of polyarteritis nodosa (PAN) (multiple aneurysms, symmetric sensorimotor peripheral neuropathy, superficial ulcers, digital necrosis, myalgia, hypertension and proteinuria). As childhood PAN is a phenocopy of adenosine deaminase 2 with a different management strategy, whole-exome sequencing was performed, which revealed a pathogenic variant in gene. The child was treated with TNF alpha inhibitors and showed improvement in the Paediatric Vasculitis Activity Score. The paper highlights the gratifying consequences of correct diagnosis with disease-specific therapy that ended the diagnostic odyssey, providing relief to the patient from debilitating symptoms and to the family from the financial burden of continued out-of-pocket health expenditure.
Topics: Humans; Polyarteritis Nodosa; Adenosine Deaminase; Female; Diagnosis, Differential; Adolescent; Exome Sequencing; Hereditary Autoinflammatory Diseases; Tumor Necrosis Factor Inhibitors; Child; Intercellular Signaling Peptides and Proteins
PubMed: 38724212
DOI: 10.1136/bcr-2023-258410 -
Clinical and Experimental Dermatology May 2024Cutaneous polyarteritis nodosa (cPN) is a necrotizing arteritis of medium-sized vessels limited to the skin. Because of its rarity and the diversity of its clinical...
Clinical and laboratory findings characterizing the need for systemic corticosteroids and nonsteroidal systemic therapies, and the predicted outcomes in cutaneous polyarteritis nodosa: A single-centre retrospective analysis.
BACKGROUND
Cutaneous polyarteritis nodosa (cPN) is a necrotizing arteritis of medium-sized vessels limited to the skin. Because of its rarity and the diversity of its clinical manifestations, there is no consensus treatment. Moreover, there are no established indicators that predict disease severity or its outcome.
OBJECTIVES
To investigate clinico-laboratory features that predict patients requiring systemic therapy, including corticosteroids, to control the disease activity.
METHODS
Thirty-six cPN patients who had not received systemic corticosteroids at the initial visit were retrospectively analysed by correlating the treatment and its response with clinico-laboratory findings.
RESULTS
The major medications administered were antiplatelet agents (63.9%), vasodilators (38.9%), and prednisolone (PSL) (36.1%). In all, 23 cases achieved remission without PSL; 5 were managed with compression therapy alone or even observation; 18 received antiplatelet monotherapy or combined with vasodilator/dapsone; 13 required PSL; 10 achieved remission with PSL monotherapy or PSL and single/multiple medications and 3 with PSL and multiple drugs failed to achieve remission and underwent limb amputation. There were more skin ulcers and an elevated peripheral white blood cell (WBC) count and erythrocyte sedimentation rate (ESR) before corticosteroid induction in patients requiring PSL. Three cases with treatment failure had a markedly elevated ESR (>50).
CONCLUSIONS
More than half of cPN can achieve remission without corticosteroids; an elevated WBC and the presence of skin ulcers predict the need for PSL; a high ESR before corticosteroid induction predicts treatment resistance, even with PSL.
PubMed: 38722089
DOI: 10.1093/ced/llae143