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The Pan African Medical Journal 2023Polyarteritis nodosa (PAN) is a systemic vasculitis affecting medium and small-sized vessels resulting in multiple organ involvement. Refractory PAN requires a different...
Polyarteritis nodosa (PAN) is a systemic vasculitis affecting medium and small-sized vessels resulting in multiple organ involvement. Refractory PAN requires a different therapeutic approach. We herein report the case of a 42-year-old male presenting a non-virus-related refractory PAN with a favorable outcome on rituximab. He presented significant weight loss, muscle weakness, peripheral axonal neuropathy, and medium-sized cutaneous vessel necrotizing vasculitis. The patient received high-dose corticosteroids and cyclophosphamide with no significant clinical improvement while developing adverse side effects such as hypertension and diabetes. Rituximab was prescribed as an alternative therapy at 1000 mg on day 0 and day 15. This allowed for complete and rapid control of disease activity with regression of cutaneous injury and substantial improvement of neurological symptoms. In conclusion, using chimeric anti-CD20 monoclonal antibodies, such as rituximab, although rarely reported in refractory non-virus-related PAN, may be an effective alternative therapy, as portrayed in our case.
Topics: Male; Humans; Adult; Polyarteritis Nodosa; Rituximab; Vasculitis; Cyclophosphamide; Drug-Related Side Effects and Adverse Reactions
PubMed: 37692987
DOI: 10.11604/pamj.2023.45.92.36496 -
BMJ Case Reports May 2021We reported a unique case with the coexistence of classic and cutaneous polyarteritis nodosa (PAN), and microscopic polyangiitis (MPA) in hepatitis virus-associated...
We reported a unique case with the coexistence of classic and cutaneous polyarteritis nodosa (PAN), and microscopic polyangiitis (MPA) in hepatitis virus-associated vasculitis. A 77-year-old Asian man presented with extremity weakness and weight loss found to have bilateral foot drop and rash on his hands and legs. Labs reveal positive for hepatitis B core antibody and perinuclear-antineutrophil cytoplasmic antibody (p-ANCA), decreased C3 and C4 levels. Skin biopsy of rash shows medium vessel vasculitis suggesting PAN. Interestingly, renal biopsy showed features of necrotising medium-sized arteritis consistent with PAN and focal crescentic glomerulonephritis consistent with MPA. The patient was treated with 1 g of solumedrol daily for 3 days, followed by oral steroids and cyclophosphamide treatment for vasculitis, and entecavir for chronic hepatitis B infection, resulting in resolution of symptoms. The patient has not had a relapse at 6 months.
Topics: Aged; Antibodies, Antineutrophil Cytoplasmic; Hepatitis B; Hepatitis B virus; Humans; Male; Microscopic Polyangiitis; Polyarteritis Nodosa
PubMed: 34011661
DOI: 10.1136/bcr-2020-240015 -
The Western Journal of Medicine Nov 1990
Review
Topics: Adult; Central Nervous System Diseases; Combined Modality Therapy; Cyclophosphamide; Drug Administration Schedule; Drug Therapy, Combination; Edema; Embolization, Therapeutic; Female; Humans; Male; Polyarteritis Nodosa; Prednisone; Rupture, Spontaneous
PubMed: 1979706
DOI: No ID Found -
British Medical Journal Dec 1955
Topics: Disease; Granuloma; Humans; Nasal Cavity; Nose Diseases; Paranasal Sinus Diseases; Polyarteritis Nodosa
PubMed: 13269926
DOI: 10.1136/bmj.2.4956.1597 -
The British Journal of Ophthalmology Jun 1978Periarteritis nodosa and thrombotic thrombocytopenic purpura in siblings is reported. In both patients a localised serous retinal detachment and lesions of the retinal...
Periarteritis nodosa and thrombotic thrombocytopenic purpura in siblings is reported. In both patients a localised serous retinal detachment and lesions of the retinal pigment epithelium had developed owing to choroidal vascular obstruction. These cases support the suggested possible relationship between the two conditions.
Topics: Adolescent; Adult; Eye; Female; Humans; Male; Pigment Epithelium of Eye; Polyarteritis Nodosa; Purpura, Thrombotic Thrombocytopenic; Retinal Detachment
PubMed: 27206
DOI: 10.1136/bjo.62.6.402 -
Scientific Reports Apr 2021Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been... (Clinical Trial)
Clinical Trial
Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3-17.5), 7.7 (3.5-17.6), and 7.0 (1.6-16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud's phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2-32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.
Topics: Adolescent; Child; Child, Preschool; Disease Progression; Female; Humans; Male; Polyarteritis Nodosa; Prognosis; Republic of Korea; Retrospective Studies; Vasculitis
PubMed: 33863937
DOI: 10.1038/s41598-021-87718-6 -
Modern Rheumatology Case Reports Jan 2023The global outbreak of coronavirus disease (COVID-19) caused by severe acute respiratory syndrome coronavirus type 2 has prompted the rapid spread and development of... (Review)
Review
The global outbreak of coronavirus disease (COVID-19) caused by severe acute respiratory syndrome coronavirus type 2 has prompted the rapid spread and development of vaccines to prevent the spread of the disease. COVID-19 vaccine has demonstrated excellent efficacy in reducing morbidity and severity of the disease, and most adverse reactions are very minor. However, some patients have been reported to develop autoimmune diseases, such as rheumatoid arthritis, myocarditis, Guillain-Barre syndrome, and vasculitis, following COVID-19 vaccination. Herein, we present a case of polyarteritis nodosa with epididymitis, following COVID-19 mRNA vaccination. The patient's initial symptoms were fever and testicular pain, and magnetic resonance imaging showed epididymitis. He was diagnosed as having polyarteritis nodosa with epididymitis and was treated with high-dose prednisolone, with a good clinical outcome.
Topics: Humans; Male; COVID-19; COVID-19 Vaccines; Epididymitis; Polyarteritis Nodosa; Vaccination
PubMed: 36305628
DOI: 10.1093/mrcr/rxac085 -
Kidney International Feb 1990
Review
Topics: Child; Child, Preschool; Glomerulonephritis; Hepatitis B; Humans; Polyarteritis Nodosa
PubMed: 1968522
DOI: 10.1038/ki.1990.32 -
BMJ Case Reports Mar 2021Polyarteritis nodosa (PAN) is a necrotising systemic vasculitis involving medium-sized and small-sized vessels. PAN limited to a single organ is rare, particularly in...
Polyarteritis nodosa (PAN) is a necrotising systemic vasculitis involving medium-sized and small-sized vessels. PAN limited to a single organ is rare, particularly in the elderly population. Herein, we present a 73-year-old-woman who developed severe abdominal pain. Mesenteric angiography showed multifocal areas of segmental dilation and narrowing of the superior mesenteric, ileocolic and right colonic arteries. Exploratory laparotomy revealed multiple areas of necrosis of the jejunum for which resection was performed. Histopathological exam disclosed mesenteric vasculitis with fibrinoid necrosis of the arterial wall with leucocytic infiltrates and haemorrhages consistent with PAN. She was started on high-dose corticosteroids with an initial good response. However, 6 months later, she developed intestinal pseudo-obstruction for which oral cyclophosphamide was started. After 5 months of cyclophosphamide therapy, she remained stable without further relapses. Our case suggests that PAN should be considered in elderly patients presenting with abdominal pain even in the absence of systemic involvement.
Topics: Abdominal Pain; Aged; Angiography; Cyclophosphamide; Female; Humans; Intestines; Polyarteritis Nodosa
PubMed: 33664043
DOI: 10.1136/bcr-2020-241431 -
California Medicine Oct 1953The collagen diseases, an ill-defined group of clinical entities, have as their basis a generalized alteration of the connective tissue, especially of its extracellular...
The collagen diseases, an ill-defined group of clinical entities, have as their basis a generalized alteration of the connective tissue, especially of its extracellular components. They include periarteritis nodosa, disseminated lupus erythematosus, dermatomyositis, scleroderma, rheumatic fever and rheumatoid arthritis. The radiological findings in a series of cases of these diseases were reviewed. In 28 cases of periarteritis, 20 cases showed some abnormal findings in the thorax. These included pleural effusions, pulmonary changes, pericardial effusions and cardiac enlargement. In 32 cases of disseminated lupus erythematosus, thoracic findings were noted in 21. They resembled the changes found in periarteritis. In some 25 cases of scleroderma, diverse radiological findings were noted. These included "cystic" changes in the lungs (one case) and pulmonary "hives." In the intestinal tract esophageal and small bowel alterations were found, both ectatic and stenotic. In the soft tissues of the "pressure areas" variable degrees of calcification were observed. Dermatomyositis is the rarest of the collagen disease group; only one autopsy-proven case is available for study. Chest x-rays taken a year before death showed slight cardiac enlargement. The lungs were clear. In acute rheumatic fever, x-ray examination may disclose pericardial or pleural effusion, and so-called rheumatic pneumonitis; the latter has no specific diagnostic features. Soft tissue swellings may develop around some of the joints. In rheumatoid arthritis, joint changes are numerous and fairly characteristic, and are followed in many cases by fibrous or bony ankylosis and deformities of considerable degree. Awareness of the commoner radiological changes in this entire group of diseases should result in earlier establishment of diagnosis, especially in the more obscure examples.
Topics: Arthritis, Rheumatoid; Collagen Diseases; Connective Tissue; Dermatomyositis; Humans; Lung; Lupus Erythematosus, Systemic; Pleural Effusion; Pneumonia; Polyarteritis Nodosa; Rheumatic Fever
PubMed: 13094539
DOI: No ID Found