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JAMA Dermatology Aug 2018In cutaneous polyarteritis nodosa (CPAN), less aggressive treatments can be selected, because CPAN is not associated with life-threatening or progressive outcomes....
IMPORTANCE
In cutaneous polyarteritis nodosa (CPAN), less aggressive treatments can be selected, because CPAN is not associated with life-threatening or progressive outcomes. Although patients with a recurrent clinical course may require additional immunosuppressive therapies, no pretreatment factors associated with a worse prognosis in CPAN have been reported.
OBJECTIVE
To identify clinical or laboratory markers associated with relapse of CPAN.
DESIGN, SETTING, AND PARTICIPANTS
This retrospective case series was performed at a dermatology clinic of a tertiary referral center in Okayama, Japan, from October 1, 2001, through April 30, 2017. Of 30 patients identified with CPAN, the 21 with histopathologic evidence of disease were eligible and enrolled in the study.
MAIN OUTCOMES AND MEASURES
The medical database was examined for sex, age at diagnosis, affected anatomical sites, type and extent of skin lesion, laboratory data, initial therapies, duration of follow-up, and current status. Relapse was defined as the first reoccurrence or new onset of cutaneous disease that required further escalation of treatment with prednisolone at a dosage of greater than 20 mg/d and/or add-on use of immunosuppressant therapy, more than 6 months after initial treatment. The pretreatment factors were statistically evaluated between the groups without and with relapse.
RESULTS
The 21 patients included 5 males and 16 females with a median age of 49 years (range, 11-74 years) at diagnosis. The median follow-up was 42 months (range, 8-374 months). Pretreatment cutaneous ulcer was significantly associated with relapse between the 2 groups (0 of 11 in the nonrelapse group vs 4 of 10 in the relapse group; χ21 = 4.67; P < .05). In the laboratory test results, significantly higher mean (SD) values were observed in the relapse group for C-reactive protein level (0.23 [2.00] vs 6.03 [3.10] mg/dL; standard error of the mean [SEM], 3.40 mg/dL; 95% CI, 0.01-10.8 mg/dL; P = .01), absolute neutrophil count (ANC) (3.4 × 103/μL [1.1 × 103/μL] vs 6.0 × 103/μL [3.2 × 103/μL]; SEM, 2.9 × 103/μL; 95% CI, 1.9 × 103/μL to 14.6 × 103/μL; P = .001), neutrophil-to-lymphocyte ratio (1.4 [0.8] vs 2.8 [0.9]; SEM, 1.2; 95% CI, 1.1-4.9; P = .002), and systemic immune-inflammation index (5.1 × 105 [3.9 × 105] vs 11.7 × 105 [7.7 × 105]; SEM, 7.3 × 105; 95% CI, 3.3 × 105 to 31.1 × 105; P = .007). The estimated 2-year cumulative relapse rate was significantly high in the patients with blood ANC of greater than 4.9 × 103/μL compared with 4.9 × 103/μL or less (9 of 10 [90%] vs 2 of 11 [18%]; 95% CI, 6%-72%).
CONCLUSIONS AND RELEVANCE
Pretreatment status of cutaneous ulcer, the serum C-reactive protein level, the blood ANC, the neutrophil-to-lymphocyte ratio, and the systemic immune-inflammation index are associated with a worse prognosis in CPAN.
Topics: Adolescent; Adult; Aged; Biomarkers; C-Reactive Protein; Child; Female; Follow-Up Studies; Humans; Japan; Lymphocytes; Male; Middle Aged; Neutrophils; Polyarteritis Nodosa; Prognosis; Recurrence; Retrospective Studies; Skin Diseases; Tertiary Care Centers; Young Adult
PubMed: 29955757
DOI: 10.1001/jamadermatol.2018.1601 -
Internal Medicine (Tokyo, Japan) 2007A 72-year-old man with cough and sputum showed esophageal wall thickening and pneumonia in chest computed tomography (CT) scan. Following endoscopy, we diagnosed reflux... (Review)
Review
A 72-year-old man with cough and sputum showed esophageal wall thickening and pneumonia in chest computed tomography (CT) scan. Following endoscopy, we diagnosed reflux esophagitis and subscribed proton pump inhibitor. The esophageal lesion, however, was intractable. We diagnosed microscopic polyangiitis (MPA) because of vasculitis symptoms, cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) in blood and no granulomatous change in the esophagus. We adopted pulse therapy of cyclophosphamide and oral prednisolone; the symptoms and esophageal lesion were markedly improved. We concluded that the esophageal lesion was an aspect of MPA. To our knowledge, this is the first report of esophageal involvement in MPA.
Topics: Aged; Antibodies, Antineutrophil Cytoplasmic; Cyclophosphamide; Diagnosis, Differential; Esophagitis; Granulomatosis with Polyangiitis; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Male; Plasma Exchange; Polyarteritis Nodosa; Prednisolone
PubMed: 17527040
DOI: 10.2169/internalmedicine.46.6115 -
The Israel Medical Association Journal... May 2014
Topics: Acne Vulgaris; Acute Kidney Injury; Anti-Bacterial Agents; Antibodies, Antineutrophil Cytoplasmic; Humans; Kidney Function Tests; Male; Minocycline; Polyarteritis Nodosa; Treatment Outcome; Withholding Treatment; Young Adult
PubMed: 24979843
DOI: No ID Found -
Medicine Jan 2002
Comparative Study
Topics: Adolescent; Adult; Age of Onset; Aged; Aged, 80 and over; Churg-Strauss Syndrome; Female; Glucocorticoids; Humans; Male; Middle Aged; Polyarteritis Nodosa; Prognosis; Retrospective Studies; Risk Factors; Survival Rate
PubMed: 11807403
DOI: 10.1097/00005792-200201000-00002 -
The Pan African Medical Journal 2021Coronary artery aneurysms are uncommon, are usually associated with atherosclerosis, and rarely involve all three major coronary arteries. Data on the optimal choice of...
Coronary artery aneurysms are uncommon, are usually associated with atherosclerosis, and rarely involve all three major coronary arteries. Data on the optimal choice of acute myocardial infarction (AMI)´s revascularization in the context of polyarteritis nodosa (PAN) is limited to case reports and is still an open question. The present report describes a rare case of a young male patient followed for PAN presenting with acute myocardial infarction (AMI). Coronary angiography revealed multiple severe aneurysmal and stenotic changes. Based on clinical feature and angiographic findings, it was strongly suspected that the AMI was a complication of his vasculitis. This case indicates that coronary artery involvement should be carefully monitored during the chronic phase of PAN. The pathophysiology of AMI in PAN patients should be kept in mind and the interventional approach must be performed according to the angiographic findings to avoid complications.
Topics: Coronary Aneurysm; Coronary Angiography; Coronary Artery Disease; Death, Sudden; Humans; Male; Middle Aged; Myocardial Infarction; Polyarteritis Nodosa
PubMed: 33912283
DOI: 10.11604/pamj.2021.38.113.27601 -
Reumatologia Clinica 2019A 47-year-old man presented with weight loss, bilateral calf pain, fever, hypertension, orchitis and oligoarthritis. Lab tests: anemia and elevated muscle enzymes....
A 47-year-old man presented with weight loss, bilateral calf pain, fever, hypertension, orchitis and oligoarthritis. Lab tests: anemia and elevated muscle enzymes. Resonance magnetic imaging: hyperintensity in gastrocnemius muscles (myositis). Histologic exam of the muscles: inflammatory infiltrate with atrophy and perifascicular regeneration. Treatment: methylprednisone (bolus) and cyclophosphamide. Muscle pain and swelling and difficulty in walking are common in panarteritis nodosa (PAN), whereas histologically demonstrated myositis is not. Even more rare is myositis as the initial presentation of this vasculitis.
Topics: Cyclophosphamide; Humans; Immunosuppressive Agents; Leg; Male; Methylprednisolone; Middle Aged; Myositis; Polyarteritis Nodosa; Prednisone
PubMed: 28755909
DOI: 10.1016/j.reuma.2017.06.010 -
The New England Journal of Medicine Mar 2014Polyarteritis nodosa is a systemic necrotizing vasculitis with a pathogenesis that is poorly understood. We identified six families with multiple cases of systemic and...
BACKGROUND
Polyarteritis nodosa is a systemic necrotizing vasculitis with a pathogenesis that is poorly understood. We identified six families with multiple cases of systemic and cutaneous polyarteritis nodosa, consistent with autosomal recessive inheritance. In most cases, onset of the disease occurred during childhood.
METHODS
We carried out exome sequencing in persons from multiply affected families of Georgian Jewish or German ancestry. We performed targeted sequencing in additional family members and in unrelated affected persons, 3 of Georgian Jewish ancestry and 14 of Turkish ancestry. Mutations were assessed by testing their effect on enzymatic activity in serum specimens from patients, analysis of protein structure, expression in mammalian cells, and biophysical analysis of purified protein.
RESULTS
In all the families, vasculitis was caused by recessive mutations in CECR1, the gene encoding adenosine deaminase 2 (ADA2). All the Georgian Jewish patients were homozygous for a mutation encoding a Gly47Arg substitution, the German patients were compound heterozygous for Arg169Gln and Pro251Leu mutations, and one Turkish patient was compound heterozygous for Gly47Val and Trp264Ser mutations. In the endogamous Georgian Jewish population, the Gly47Arg carrier frequency was 0.102, which is consistent with the high prevalence of disease. The other mutations either were found in only one family member or patient or were extremely rare. ADA2 activity was significantly reduced in serum specimens from patients. Expression in human embryonic kidney 293T cells revealed low amounts of mutant secreted protein.
CONCLUSIONS
Recessive loss-of-function mutations of ADA2, a growth factor that is the major extracellular adenosine deaminase, can cause polyarteritis nodosa vasculopathy with highly varied clinical expression. (Funded by the Shaare Zedek Medical Center and others.).
Topics: Adenosine Deaminase; Adolescent; Age of Onset; Child; Child, Preschool; Exome; Female; Genes, Recessive; Georgia (Republic); Humans; Infant; Intercellular Signaling Peptides and Proteins; Jews; Male; Middle Aged; Mutation; Pedigree; Polyarteritis Nodosa; Turkey
PubMed: 24552285
DOI: 10.1056/NEJMoa1307362 -
World Journal of Gastroenterology Jan 2015Classic polyarteritis nodosa (PAN) that targets medium-sized muscular arteries and microscopic polyangiitis (MPA), characterized by inflammation of small-caliber vessels... (Review)
Review
Classic polyarteritis nodosa (PAN) that targets medium-sized muscular arteries and microscopic polyangiitis (MPA), characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA), are distinct clinicopathological entities of systemic vasculitis. A 66-year-old woman presented with fever, cholestasis and positive MPO-ANCA. Radiological examination showed a pancreatic mass compressing the bile duct. Therefore, we performed pancreatoduodenectomy. Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels, spared arterioles or capillaries in the pancreas, a finding consistent with PAN. Unexpectedly, renal biopsy revealed small-caliber vasculitis and glomerulonephritis, supporting MPA. The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles. Its diagnosis is challenging because no reliable clinico-radiological findings have been observed. Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory. Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA.
Topics: Aged; Antibodies, Antineutrophil Cytoplasmic; Biomarkers; Biopsy; Diagnosis, Differential; Female; Fibrosis; Glomerulonephritis; Humans; Immunosuppressive Agents; Kidney; Microscopic Polyangiitis; Necrosis; Pancreas; Pancreatic Diseases; Pancreaticoduodenectomy; Peroxidase; Polyarteritis Nodosa; Predictive Value of Tests; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 25624739
DOI: 10.3748/wjg.v21.i3.1014 -
Medicine Jul 2023We report the case of a patient who initially presented with peripheral neuropathy and myositis without typical organ involvement, such as the kidneys, skin, or...
RATIONALE
We report the case of a patient who initially presented with peripheral neuropathy and myositis without typical organ involvement, such as the kidneys, skin, or gastrointestinal system, but was ultimately diagnosed with polyarteritis nodosa (PAN).
PATIENT CONCERNS
A 62-year-old man presented with radicular pain in his right lower extremity. One week later, he complained of right ankle motor weakness and pain in the right posterior thigh, which led to admission. After 6 weeks of hospitalization, he newly experienced pain in his right testicle and anterior thigh.
DIAGNOSIS
The patient was initially diagnosed with polymyositis combined with sciatic neuropathy using magnetic resonance imaging, electrodiagnostic tests, and muscle biopsy. However, with the emergence of other systemic symptoms such as testicular pain, vasculitis was suspected, and the patient was reclassified as PAN using the 2007 European Medicines Agency algorithm and the American College of Rheumatology criteria.
INTERVENTIONS
The patient was treated with glucocorticoids for more than 6 months, and antiviral medication was prescribed to prevent hepatitis B virus reactivation.
OUTCOMES
The patient's radicular pain and pain in the right anterior and posterior thighs and testicle improved, and there were no signs of recurrence.
LESSONS
In patients presenting with radicular and focal muscle pain, it is crucial to consider the potential for PAN, as observed in this case report.
Topics: Male; Humans; Middle Aged; Polyarteritis Nodosa; Peripheral Nervous System Diseases; Polymyositis; Testis; Muscle Weakness; Pain
PubMed: 37478214
DOI: 10.1097/MD.0000000000034335 -
CEN Case Reports May 2021We present the case of a 67-year-old man in good health with perirenal hematoma due to a ruptured arterial aneurysm in the kidney. The patient developed weight loss,...
We present the case of a 67-year-old man in good health with perirenal hematoma due to a ruptured arterial aneurysm in the kidney. The patient developed weight loss, muscle weakness, multiple mononeuropathy, hypertension, anemia, renal insufficiency, and multiple lacuna infarctions about a month ago. He was admitted to the hospital due to worsening of his symptom. After admission, severe right-flank pain suddenly occurred; he was then transferred to our hospital. Renal angiography revealed bilateral multiple microaneurysms, and the patient was diagnosed with polyarteritis nodosa based on the clinical, radiographic, and histological findings. We performed selective coil embolization to the ruptured aneurysm and administered oral prednisolone along with intravenous methylprednisolone pulse therapy. Cyclophosphamide pulse therapy was also given. The treatment improved clinical and laboratory findings and achieved clinical remission. Selective coil embolization to the bleeding aneurysm of polyarteritis nodosa was minimally invasive and promptly effective. Immunosuppressants proved useful in the regulation of disease activity and the aneurysm.
Topics: Aged; Aneurysm, Ruptured; Hematoma; Humans; Kidney Diseases; Male; Polyarteritis Nodosa; Renal Artery
PubMed: 33175365
DOI: 10.1007/s13730-020-00552-z