Manifestation Of Microscopic Polyangiitis Research Articles

Ураження суглобів при ANCA-асоційованих системних васкулітах Повідомлення І. Мікроскопічний поліангіїт

Артикулярний синдром є однією з основних ознак мікроскопічного поліангіїту (МПА), на частку якого в деяких регіонах земної кулі припадає понад 90 % усіх системних васкулітів (СВ), що пов’язані з антинейтрофільними цитоплазматичними антитілами (ANCA). Було встановлено, що за 5 років після дебюту МПА починають формуватися прогресуюче зменшення параартикулярної (епіфізарної) мінеральної щільності кістки й деструкція суглобів. Дуже багато питань, пов’язаних із характером перебігу суглобової патології при окремих ANCA-CB, вимагають подальшого вивчення. Мета: оцінити ураження суглобів у хворих на МПА та взаємозв’язки з екстраартикулярною патологією. Матеріал і методи. Під спостереженням перебували 89 хворих на МПА віком 18–78 років, серед яких було 44 % чоловіків і 56 % жінок. Тривалість хвороби від перших ознак її маніфестації становила 7 років. Гострий або підгострий перебіг МПА встановлено в 27 % випадків, у решти — хронічний; І ступінь активності патологічного процесу констатовано в 10 % спостережень, ІІ — в 32 %, ІІІ — в 58 %. Результати. Ураження суглобів спостерігається в 60 % від числа хворих на МПА, що у 40 % випадків перебігає у вигляді олігоартриту, залежить від наявності патології скелетних м’язів, пневмопатії і периферійної нейропатії. Клінічні, рентгенологічні та сонографічні ознаки артикулярної патології при МПА значно пов’язані з віком пацієнтів, тривалістю захворювання, ураженням серця, нирок і шкіри. У патогенетичних побудовах артропатії у хворих на МПА беруть участь С-реактивний протеїн (CRP) та циркулюючі імунні комплекси (CIC), а слабке значення має характер ANCA (антитіла до мієлопероксидази і протеїнази-3), при цьому певну прогнознегативну значущість щодо тяжкості суглобової патології мають високі параметри в крові ревматоїдного фактора (RF). Висновок. Прогнознегативними критеріями щодо розвитку патології суглобів у хворих на МПА є показники в крові: CRP > 20 мг/л і CIC > 130 ум.од., а значення RF > 60 МО/мл вважаються чинниками ризику тяжкого перебігу артропатії.

Recognizing Central Nervous System Involvement as a Progressive Feature of Microscopic Polyangiitis: A Diagnostic Dilemma

Microscopic polyangiitis is an idiopathic autoimmune disease characterized by systemic vasculitis predominantly of small-caliber blood vessels with antineutrophilic cytoplasmic antibodies (ANCA), and it can have a variable presentation. 1 Chung S.A. Seo P. Microscopic polyangiitis. Rheum Dis Clin N Am. 2010; 36: 545-558 Abstract Full Text Full Text PDF PubMed Scopus (82) Google Scholar We present the case of a 57-year-old male smoker with central nervous system-related symptoms of left facial droop, dysarthria, and worsening left upper-extremity weakness and numbness due to multiple strokes as a manifestation of microscopic polyangiitis.

Clinical characteristics of 15 cases of microscopic polyangiitis associated with alveolar hemorrhage

To investigate the clinical features of patients with microscopic polyangiitis (MPA) and alveolar hemorrhage (AH), so as to raise the physicians' understanding of this condition. The clinical data of 15 MPA patients combined with AH, who were hospitalized in Peking Union Medical College Hospital from January 2008 to January 2014, were retrospectively analyzed. These 15 subjects accounted for 18.1% of all MPA patients, including 4 males and 11 females with mean age of (59.4 ± 17.9) years old and mean disease duration of 7.5 (1-48) months. Hemoptysis or bloody sputum (14/15) and dyspnea (11/15) were predominant pulmonary manifestations. Hypoxemia appeared in 6 patients (40.0%). An average 8-46 (22.7 ± 4.4) g/L drop of hemoglobin level was seen in all 15 patients. Imaging revealed new bilateral infiltrations or opacities in 13 cases, and bloody bronchoalveolar lavage fluid were found in 6 cases. Compared with MPA patients without AH, the incidence of myalgia [2/15 vs 50.0%(34/68); P=0.009, OR=0.154] and hemoglobin level [(83.2 ± 21.0) g/L vs (102.5 ± 18.7) g/L; P=0.001] were statistically lower in MPA patients with AH. There were no statistical differences in age, sex, interstitial lung disease, renal involvement, peripheral neuropathy and the five factor score between two groups. The proportion of patients who received corticosteroid pulse treatment in MPA with AH group was significantly higher than that of MPA patients without AH [9/15 vs 19.1%(13/68); P=0.003, OR=6.346]. After treatment, 13 cases achieved remission, while 2 died. As an important pulmonary manifestation of MPA, AH could be insidious but fatal. The treatment with steroid, cyclophosphamide and plasma exchange could improve the prognosis.

We Present a Case of Cardiac and Ear Involvement as Atypical Manifestations of Microscopic Polyangiitis

We Present a Case of Cardiac and Ear Involvement as Atypical Manifestations of Microscopic Polyangiitis

Microscopic polyangiitis associated with antineutrophil cytoplasmic antibodies: clinical features

To study the clinical features of early- and extended-stage microscopic polyangiitis (MPA) and its outcomes on the basis of a long-term follow-up in a rheumatologist's practice. The clinical features of early- and extended-stage MPA were studied in detail and the premorbid background and possible precipitating factors were analyzed in 70 patients with MPA and the proven hyperproduction of antineutrophil cytoplasmic antibodies (anti-proteinase-3 (anti-PR3) antibodies in 55% and anti-myeloperoxidase (anti-MPO) antibodies in 45%) who had been followed up for more than a year. There is evidence for the nosological unity of the two immunological types of MPA associated with anti-PR-3 or anti-MPO antibodies. MPA has been demonstrated to be an aggressive, polysyndromic disease prone to recurrences (52%), the typical manifestation of which is glomerulonephritis (94%) that is rapidly progressive in every four cases and accompanied by hemorrhagic alveolitis (69%) and involvement of other organs. ENT organs and lungs have been noted to be commonly involved in early-stage MPA, which was observed in 61% of the patients in the premorbid period, and to become the first manifestation of MPA (63%) concurrent with body temperature rises (64%), arthralgia or arthritis (41%). Respiratory tract involvement in MPA may be asymptomatic. Anti-PR-3-associated MPA may manifest itself more aggressively and in the first 2 years it is characterized by a poorer prognosis than of anti-MPO-associated MPA (survival rates, 82 and 94%, respectively; p = 0.04). With time, the differences were levelled off; recurrences in the patients with anti-PR-3 and anti-MPO develop equally frequently and proceed showing the similar clinical picture; the survival curves converge by age 3. Anti-MPO-associated MPA is characterized by the highest rate of lung involvement in the clinical phase of the disease (61%) and by a propensity to develop hemorrhagic alveolitis, diffuse interstitial (22%) or circumscribed pulmonary fibrosis in the outcome. CONCLUSION. The findings emphasize how important to diagnose MPA early and to prescribe long-term active treatment using the entire current arsenal of medications as soon as possible until severe injury to organs and systems develops. To specify regularities in the development of MPA may be of value for the better diagnosis of the disease and the further elaboration of optimal treatment policy.

Microscopic polyangiitis associated with pulmonary fibrosis.

Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis characterised by inflammation of the small blood vessels, the absence of granulommas on histopathological specimens, with few or no immune deposits and the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCAs). The classic pulmonary manifestation is diffuse alveolar haemorrhage (DAH), but its association with pulmonary fibrosis (PF) has been increasingly reported and may be the first manifestation of MPA. Our aim was to evaluate MPA patients with PF and compare their characteristics and evolution to those of MPA patients without PF. We conducted a retrospective review of MPA patients followed in our hospital over a 15-year period. They were divided into two subgroups, with PF (MPA-PF) and without PF (MPA-non PF), and their clinical and functional features were compared. Nine of the 28 patients were classified as MPA-PF (32%). This subgroup showed significantly more respiratory symptoms and higher mortality than MPA-non PF subgroup. The most frequent chest computed tomographic pattern of PF was usual interstitial pneumonia. PF preceded other manifestations of vasculitis in five patients and occurred simultaneously in the remaining four. During the follow-up period, four deaths were reported in the MPA-PF subgroup. No deaths were registered in the MPA-non PF subgroup. We found a high prevalence of MPA-PF patients (32%), most of whom had a poor outcome and PF was often the first manifestation of the disease.

Posterior Reversible Encephalopathy Syndrome in a Patient with Microscopic Polyangiitis (P01.127)

Objective: To discuss neurologic manifestations of microscopic polyangiitis (MPA) vasculitis. Background Central nervous system manifestations of MPA are uncommon. Only two cases of posterior reversible encephalopathy syndrome (PRES) in patients with MPA have been reported. Design/Methods: A case report. Setting: A tertiary care center. Patient: A 69-year old man with chronic kidney disease of unknown etiology came to the ED with new-onset right foot numbness. He was febrile and normotensive. Labs revealed a leukocytosis, anemia and an elevated creatinine. Urinalysis showed 21-30 wbcs, rbcs too numerous to count, 3+ protein, and 3+ blood. CT head was unremarkable. A diagnosis of MPA vasculitis was made based on serum positivity for myeloperoxidase (MPO)-ANCA and renal biopsy. He received steroids and one dose of cyclophosphamide but had a complicated hospital course, with respiratory distress requiring intubation and renal failure requiring CVVH followed by hemodialysis. On Day 28 he went from interactive and able to follow commands to acutely delirious. Hours later he had a generalized seizure and went into status epilepticus. CT scan revealed extensive edema bilaterally in the posterior portions of both hemispheres, raising concern for PRES. CT on hospital day 35 was again consistent with PRES. The patient remained intubated and sedated and was unable to recover. On Day 43, the patient9s family opted for terminal extubation. Results: Our diagnosis was PRES as indicated by the CT scans. Conclusions: The pathophysiology of PRES in patients with MPA vasculitis is unclear. Endothelial damage might predispose to PRES by increasing blood-brain barrier permeability. Our patient had renal failure and was immunosuppressed, which may have also contributed to his development of PRES. MPA itself, the renal failure that it may induce, and the agents used to treat it all may be associated with the development of PRES. Disclosure: Dr. Minen has nothing to disclose. Dr. Gold has nothing to disclose. Dr. Badjatia has nothing to disclose.

Sudden onset blindness treated with pulse steroid therapy in a patient with microscopic polyangiitis

AbstractWe report a 71-year-old male with microscopic polyangiitis (MPA) who developed sudden-onset, progressive, bilateral visual loss associated with a relapse of MPA symptoms. The patient was referred to our hospital, and treated with intravenous pulse steroid therapy and high-dose oral prednisolone. Although the right eye remained vision deficient, visual acuity in the left eye recovered. Ocular manifestations of MPA are quite uncommon. This case emphasizes the necessity of early detection and initiation of prompt therapy where ocular manifestations of MPA occur.

Mikroskopische Polyangiitis als ätiologischer Faktor der Lungenfibrose – eine bekannte, doch häufig spät diagnostizierte Ursache

Lung fibrosis as first symptomatic manifestation of microscopic polyangiitis (MPA) is rare. It is characterized by progressive exertional dyspnea, radiologic detection of reticular shadowing, and increased titers of MPA-associated autoantibodies. This case study shall remind of the rare treatable cause of lung fibrosis. A 78-year-old male patient with long-standing exertional dyspnea and typical chest radiograph appearances of advanced lung fibrosis is presented. The finding of MPA autoantibodies in laboratory tests was decisive to confirm the diagnosis of MPA, and a therapy adapted to the disease severity could be initiated. An early diagnosis of MPA and a fast onset of therapy are important, because the influence of an already existent lung fibrosis is uncertain.