Involvement In Polyarteritis Nodosa Research Articles

Characteristic Imaging Patterns of Muscle Involvement in Polyarteritis Nodosa: Case Report and Review of the Literature

Objective: Polyarteritis nodosa (PAN) is a rare disease with complex clinical manifestations that are difficult to diagnose. Imaging diagnoses of previously reported patients have focused on vascular manifestations. Magnetic resonance imaging (MRI) has been used to detect muscle involvement in PAN. Here, we reviewed imaging findings pertaining to muscle involvement in patients with PAN. Methods: Twelve articles concerning muscle involvement in PAN were published during the period 1980–2020; 21 patients, including our patient, were examined in this study. Results: Across the published articles, the male to female ratio was 1:1, the mean patient age was 40.76 ± 18.28 years, and there were 17 patients with calf involvement and 3 with thigh involvement. The T1WI and T2WI findings were both isointense in one patient, and the T1WI findings alone were isointense in seven patients. The T1WI findings were slightly hyperintense in five patients, and no T1WI images were available for the remaining seven patients. The T2WI signal was diffusely hyperintense in 10 patients, and “patchy villous hyperintense” in 9 patients. Among the 12 patients with enhanced images, most exhibited diffuse or cotton-like enhancement, while some showed involvement of the fascia and periosteum. The comprehensive imaging analysis of our patient included muscle and blood vessel MRI and computed tomography (CT) examinations. Our patient’s disease involved the calves and thighs, with T1WI isointensity and T2WI patch-like hyperintensity, as well as cotton-like enhancement centered on blood vessels. Computed tomography angiography (CTA) and magnetic resonance angiography (MRA) examinations of the lower limbs showed beadlike changes in the arterial branches, and the lower leg arterial branches were obvious. Head MRA revealed stenosis and occlusion of the right middle cerebral artery. Additionally, the superior mesenteric artery was locally dilated around a tumor, with the greatest width being approximately 9 mm. Cerebral perfusion analysis indicated cerebral blood flow (CBF) was lower in the right cerebellar hemisphere.Conclusions: PAN should be considered in the presence of patchiness or diffuse muscle signal changes on MRI of the lower leg (or thigh), followed by vessel-centered cotton-like enhancement accompanied by fascial or periosteal enhancement. Our findings suggest that systemic examination of small and medium arteries in patients with PAN can aid early prevention and treatment.

Characteristics and Outcomes of Coronary Artery Involvement in Polyarteritis Nodosa

BackgroundCoronary artery involvement is a severe but uncommon manifestation of polyarteritis nodosa (PAN), so clinicians have little knowledge of it. Our aim was to investigate the clinical characteristics, risk factors and outcomes of patients with PAN complicated with coronary artery lesions. MethodsData from 145 patients with PAN who were admitted to Peking Union Medical College Hospital from January 2000 to September 2019 were retrospectively collected. ResultsNineteen patients (13.1%) had coronary artery lesions due to PAN. The age at the onset of PAN was 32.3 ± 11.8 years. There were no significant differences in common risk factors for coronary arterial atherosclerosis between the patients with coronary artery involvement and those without. Affected branches of the coronary arteries were left anterior descending branch (15 patients), right coronary artery (14 patients), and left circumflex branch (9 patients). Eleven of the 19 patients exhibited multivessel lesions. Multivariate logistic regression analysis showed that celiac artery involvement (odds ratio [OR] 3.722, 95% confidence interval [CI] 1.115-12.427; P = 0.033) and new-onset hypertension (OR 6.668, 95% CI 1.936-22.961; P = 0.003) were risk factors for coronary artery involvement in patients with PAN. Stent placement was performed for 2 patients, and in-stent restenosis occurred in 1 of those patients a year later. ConclusionsPAN with coronary artery involvement exhibits more combined involvement of arteries of other organs and more severe diseases. PAN should be considered when treating young adults with an unknown origin of coronary artery lesions. In addition to systemic immunosuppressive treatment, other measures including antiplatelet and anticoagulation therapy should be initiated; however, determining the optimal time to perform procedures such as intervention or surgery is still challenging.

Chronic Abdominal Pain in a 47-Year-Old Woman

Question: A 47-year-old woman presented for a second opinion for a two month history of unexplained epigastric pain and a 5 kg weight loss. She described the pain as intermittent and exacerbated by meals. She denied other accompanying gastrointestinal symptoms including nausea, vomiting, or changes in her bowel habits. Her past medical history was significant for chronic sinusitis and a prior Cesarean section. Physical examination revealed moderate tenderness to palpation over the lower sternal and epigastric region without associated rebound tenderness or guarding. The remainder of the physical examination including vital signs was unremarkable. An evaluation prior to presentation including computed tomography (CT) of the abdomen and pelvis, esophagogastroduodenoscopy, and hepatobiliary scan had been unrevealing. Laboratory investigation was significant for elevated C-reactive protein and erythrocyte sedimentation rate (280 mg/L and 65 mm/1h, respectively). CT angiography demonstrated patent mesenteric vasculature and subtle beading of the right and left renal arteries (Figure A, arrows). In the setting of ongoing abdominal pain, a conventional mesenteric and renal angiography was performed and showed multiple small- to medium-sized branch aneurysms, areas of luminal irregularity, and occlusive disease (Figures B and C, arrows). Additional laboratory studies revealed normal antinuclear antibodies, cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), proteinase 3 antibodies (PR-3), and cryoglobulin levels. Which of the following diagnoses likely accounts for the patient’s abdominal pain?A.Microscopic polyangiitisB.Granulomatosis with polyangiitisC.Polyarteritis nodosaD.Eosinophilic granulomatosis with polyangiitis Look on page 628 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image(s) to Practical Teaching Cases. The correct answer is C. The radiographic findings of aneurysms involving small- to medium-sized arteries on angiography (Figures B and C) in combination with normal laboratory testing for ANCA, PR-3, and cryoglobulin levels in the setting of chronic abdominal pain and weight loss are most consistent with a diagnosis of PAN. In this case, following negative testing for hepatitis B (HBV) and C virus (HCV), she was initiated on combination therapy with a prednisone taper and intravenous cyclophosphamide and achieved disease remission with resolution of her symptoms after six months of therapy. Although vasculitides are infrequently encountered in clinical practice, gastrointestinal (GI) involvement by these conditions is common and often manifest with non-specific symptoms including abdominal pain, nausea, vomiting, diarrhea, and bleeding. This case highlights how radiographic features and laboratory data may be used to distinguish between vasculitic entities. Microscopic polyangiitis (answer A) is an ANCA-associated vasculitis (AAV) characterized by involvement of small arteries and veins which conflicts with the findings in this case. Histology-confirmed features of necrotizing granulomatous vasculitis facilitate a diagnosis of granulomatosis polyangiitis (answer B) which is also frequently associated with c-ANCA and PR3 antibodies. Eosinophilic granulomatosis with polyangiitis (answer D), or Churg-Strauss syndrome, while an important consideration in patients with unexplained abdominal pain, is unlikely in the absence of concomitant asthma and peripheral eosinophilia and would not account for the described radiographic features. PAN represents a non-AAV capable of predominately affecting the muscular medium-sized arteries of any organ system except the lungs.1De Virgilio A. Greco A. Magliulo G. et al.Polyarteritis nodosa: A contemporary overview.Autoimmun Rev. 2016; 15: 564-570Crossref PubMed Scopus (121) Google Scholar GI manifestations of this condition are variable but abdominal pain, often secondary to ischemia resulting from mesenteric arteritis2Ebert E.C. Hagspiel K.D. Nagar M. et al.Gastrointestinal involvement in polyarteritis nodosa.Clin Gastroenterol Hepatol. 2008; 6: 960-966Abstract Full Text Full Text PDF PubMed Scopus (56) Google Scholar is the most common.3Levine S.M. Hellmann D.B. Stone J.H. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients.Am J Med. 2002; 112: 338-349Abstract Full Text Full Text PDF Scopus (122) Google Scholar While PAN is a clinical diagnosis and does not require histologic confirmation, its association with several infections including HBV and HCV infection is well-recognized and should prompt evaluation for these conditions if identified de novo. Treatment is tailored toward disease severity and the presence of concomitant disease processes. Induction of remission in moderate to severe disease is usually achieved with combination glucocorticoids and cyclophosphamide,1De Virgilio A. Greco A. Magliulo G. et al.Polyarteritis nodosa: A contemporary overview.Autoimmun Rev. 2016; 15: 564-570Crossref PubMed Scopus (121) Google Scholar although antiviral therapy is essential in HBV and HCV infection while limiting immunosuppressive medications. CME Exam 2: Chronic Abdominal Pain in a 47-Year-Old WomanGastroenterologyVol. 155Issue 3Preview Full-Text PDF

Occult Renal Involvement in Polyarteritis Nodosa: The Role of Renal Angiography

Occult Renal Involvement in Polyarteritis Nodosa: The Role of Renal Angiography

Bilateral Subcapsular and Perinephric Hemorrhage as the Initial Presentation of Polyarteritis Nodosa

Polyarteritis nodosa is a systemic vasculitis of medium and small-sized arteries associated with aneurysm formation. Aneurysms may be complicated by thrombosis, rupture and hemorrhage. Kidney involvement in polyarteritis nodosa can rarely be complicated by Wunderlich syndrome which is characterized by acute onset of spontaneous, nontraumatic subcapsular and perirenal hematomas. A 22-year-old woman with mental retardation due to childhood cytomegalovirus infection presented with flank pain, weight loss and fever, and was found to have bilateral subcapsular and perinephric hematomas. Spontaneous bilateral renal hemorrhage as the initial manifestation of polyarteritis nodosa is rare, and it can be associated with delays in diagnosis and treatment.

Pulmonary Capillaritis In A Classical Polyarteritis Nodosa

Introduction: Pulmonary involvement in polyarteritis nodosa is very uncommon. A 65 year old woman with well documented, polyarteritis nodosa was found to have pulmonary capillaritis presenting as alveolar hemorrhage. Patient responded well to the treatment for alveolar hemorrhage.Methods: We report a case of polyarteritis nodosa with diffuse alveolar hemorrhage due to pulmonary capillaritis.Discussion: We describe a case of Polyarteritis nodosa who presented with pulmonary hemorrhage due to pulmonary capillaritis and hence might represent an overlap of polyarteritis nodosa with Microscopic Polyangiitis (MPA) or other unclassified collagen vascular disease.Conclusion: We conclude that pulmonary capillaritis can coexist with classical polyarteritis nodosa.

Gastrointestinal involvement in polyarteritis nodosa (1986–2000):: Presentation and outcomes in 24 patients

PurposeGastrointestinal involvement in polyarteritis nodosa carries a poor prognosis. A 1982 review from our institution reported acute abdominal syndromes in 31% of patients with polyarteritis nodosa, and that all 5 patients with acute surgical abdomens died. We reviewed our more recent experience to determine if outcomes have changed since. Subjects and methodsWe reviewed the records of all patients with polyarteritis nodosa in our vasculitis database between 1986 and 2000. Inclusion criteria were a diagnosis of polyarteritis nodosa, symptoms or signs of gastrointestinal involvement, and either a mesenteric angiogram consistent with polyarteritis nodosa or histopathologic proof of a medium-vessel vasculitis. We calculated a prognostic (5 factor) score for all patients. ResultsWe identified 24 patients with polyarteritis nodosa who had gastrointestinal involvement during their illness. Thirteen (54%) of the patients developed acute surgical abdomens, 3 of whom died (P = 0.02 by comparison with the historical cohort). Mean (± SD) prognostic scores were higher among patients in the acute abdomen group compared with those who did not have acute abdominal syndromes (1.7 ± 0.9 vs. 0.6 ± 0.7, P = 0.002), corresponding with the observed mortality in these groups. ConclusionGastrointestinal involvement occurs commonly in polyarteritis nodosa and carries a poor prognosis. Compared with a historical cohort at our institution, mortality from this complication may have decreased, perhaps because of earlier diagnosis.

Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children.

Renal involvement is common in childhood polyarteritis nodosa (PAN). We report a retrospective analysis of the presentation and clinical course of 26 patients with PAN and renal involvement. The mean age was 9.3 years (range 1-14 years) and there were 12 boys and 14 girls. Renal symptoms at presentation were as follows: 3 had isolated proteinuria, 9 had nephritic syndrome, 2 had nephritic and nephrotic components, and 10 had renal failure with one of the above features. Two patients with isolated hypertension were diagnosed by angiography and classified as classical PAN. Patients either received prednisone p.o. alone (n=9), or prednisone plus cyclophosphamide p.o. (n=11), or pulse steroids with prednisone p.o. and cyclophosphamide (n=2); 4 did not receive any treatment. Patients who were given cyclophosphamide had a significantly better outcome than those who did not. We suggest that oral cyclophosphamide therapy and corticosteroids are effective in the treatment of PAN. The overall 1-year and 5-year survival rates of the patients were 72.5% and 60%, respectively. In conclusion, renal disease is a serious manifestation of PAN necessitating prompt and aggressive treatment.

Localisation utérine d'une périartérite noueuse révélée par une fièvre au long cours

We report the case of an 81 year-old woman admitted because of declining general health, fever and elevated sedimentation rate. Identification of a pelvic mass on the computed tomographic scan led to perform hysterectomy with a working diagnosis of ovarian malignancy. Histological examination revealed typical lesions of polyarteritis nodosa of uterus and fallopian tube vessels. Diffuse process of the vasculitis was suggested by persistence of fever and elevated erythrocyte sedimentation rate after hysterectomy. Treatment consisting of high-dose corticosteroids and pulses of cyclophosphamide resulted in prompt clinical and biological improvement. We discuss the incidence, the clinical features and the localized or diffuse nature of genital involvement in polyarteritis nodosa and other vasculitis.

Polyarteritis nodosa presenting as acute blindness

Polyarteritis nodosa is a vasculitic disease commonly involving coronary arteries, mesenteric arteries, kidneys, muscles, and vasa nervorum. Central nervous system involvement in polyarteritis nodosa is unusual and tends to occur later in the course of disease. We report the case of a 23-year-old woman who presented to our emergency department with acute loss of vision. Her evaluation led to a diagnosis of polyarteritis nodosa. We present what may be the first published case report of polyarteritis nodosa presenting as acute bilateral visual loss.

Successful management of catastrophic gastrointestinal involvement in polyarteritis nodosa

A patient with polyarteritis nodosa developed necrotizing enterocolitis, as indicated by pneumatosis intestinalis seen on computed tomographic scans of the abdomen. Despite immunosuppressive therapy and concomitant resolution of the intramural and portal venous gas and general clinical improvement, on 2 occasions (between 20 and 30 days later) the patient developed bowel infarctions and perforations that necessitated bowel resection. Leaks developed at anastomotic sites, but were not closed surgically. However, these sites and the lower quadrants of the abdomen were drained, and the patient was given total parenteral nutrition. Over a 2-month period the patient completely recovered from this nearly always fatal gastrointestinal complication of polyarteritis nodosa. The medical, surgical, and radiographic approach we used may be applicable to the management of similar cases in the future.

Polyarteritis nodosa with new bone formation.

1. A case of polyarteritis nodosa is reported, the presenting manifestation of which was subperiosteal new bone formation in both legs. 2. The effect of cortisone on the symptoms is recorded. 3. The histology is reported after biopsy of bone and adjacent tissue. 4. Skeletal involvement in polyarteritis nodosa is believed to be hitherto unrecorded.