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Pulmonary Arteritis


Presentation

  • A 45-year-old female was presented with progressive dyspnea and bilateral leg edema. Pulmonary angiography revealed total occlusion of the right pulmonary artery and significant stenosis of the left pulmonary artery.[ncbi.nlm.nih.gov]
  • Nuclear atypia was not present and no microorganisms were seen.[journalpulmonology.org]
  • Nevertheless, it presented as the initial isolated presentation is seldom seen.[scielo.br]
  • The authors present a case involving a 48-year-old First Nations man presenting with a six-month history of exertional dyspnea and severe stenosis of the left pulmonary artery, who was ultimately diagnosed with pulmonary arteritis related to large vessel[ingentaconnect.com]
Fatigue
  • She had also experienced recurrent syncope on exertion and complained of occasional burning chest pain and vague fatigue.[karger.com]
  • These mostly include chest pain, cough, signs of pulmonary hypertension such as dyspnea, fatigue, angina, syncope [ 28 – 31 ], and hemoptysis [ 32, 33 ], with rare cases of pulmonary hemorrhage [ 34 – 36 ].[thoracickey.com]
  • About 50% of patients report constitutional symptoms such as fever, malaise, night sweats, weight loss, fatigue, and/or arthralgias. Repetitive arm movements and sustained arm elevation may cause pain and fatigue.[msdmanuals.com]
  • , and evidence of cardiac involvement were good predictors for either death or major event on follow-up, which help the prognosis assessment and elective interventions. 3 Case report An 18-year-old yellow Asian female patient had been suffering from fatigue[scielo.br]
  • Symptoms: Some of the early signs of cor pulmonale include constant cough, difficulty breathing, fatigue and weakness.[medigoo.com]
Leg Edema
  • A 45-year-old female was presented with progressive dyspnea and bilateral leg edema. Pulmonary angiography revealed total occlusion of the right pulmonary artery and significant stenosis of the left pulmonary artery.[ncbi.nlm.nih.gov]
Soft Tissue Mass
  • Fig. 2 Ultrasound-guided transbronchial fine-needle aspiration of the soft-tissue mass around the right pulmonary artery contains exclusively normal lymphatic tissue cells with anthracotic pigment containing histiocytes (arrows).[karger.com]
Epilepsy
  • He had a history of pulmonary tuberculosis at the age of 13, Sydenham's chorea, epilepsy, granulomatous dermatitis, recurrent uveitis of both eyes and post-phacoemulsification of left eye.[journalpulmonology.org]
Rales
  • […] snap僧帽弁開放音 mitral regurgitation (MR)僧帽弁逆流[症] mitral stenosis (MS) 僧帽弁狭窄[症] mitral valve 僧帽弁 mitral valve area僧帽弁口面積 mitral valve prolapse (MVP)僧帽弁逸脱[症] mitral valve replacement僧帽弁置換術 Mobitz type I blockモビッツI型ブロック Mobitz type II blockモビッツII型ブロック moist rale[tokyo-med.ac.jp]
Painful Cough
  • These mostly include chest pain, cough, signs of pulmonary hypertension such as dyspnea, fatigue, angina, syncope [ 28 – 31 ], and hemoptysis [ 32, 33 ], with rare cases of pulmonary hemorrhage [ 34 – 36 ].[thoracickey.com]
Choking
  • […] systemic manifestation of the disease. 11 Involvement of the respiratory system has been reported with a frequency ranging from 9% to 31% of GCA patients. 4 Respiratory symptoms described include cough, 9,11,13 dyspnoea, 11 sore throat, hoarseness, choking[journalpulmonology.org]
Aphthous Ulceration
  • ulceration or scarring, observed by physician or patient Eye lesions Anterior uveitis or posterior uveitis, or cells in vitreous on slit lamp examination or retinal vasculitis observed by ophthalmologist Skin lesions Erythema nodosum observed by physician[thoracickey.com]
Ejection Murmur
  • Auscultation of the lung was normal, while cardiac auscultation revealed a harsh 3/6 systolic ejection murmur with radiation to the back. A diagnostic workup was initiated.[karger.com]
Shoulder Pain
  • He also complained of general malaise, anorexia, weight loss of 4 kg, muscle pain predominating in upper limbs and bilateral shoulder pain and jaw claudication within the last 2 months. He had neither respiratory nor genitourinary tract complaints.[journalpulmonology.org]
Dermatitis
  • He had a history of pulmonary tuberculosis at the age of 13, Sydenham's chorea, epilepsy, granulomatous dermatitis, recurrent uveitis of both eyes and post-phacoemulsification of left eye.[journalpulmonology.org]
Chorea
  • He had a history of pulmonary tuberculosis at the age of 13, Sydenham's chorea, epilepsy, granulomatous dermatitis, recurrent uveitis of both eyes and post-phacoemulsification of left eye.[journalpulmonology.org]

Workup

  • A diagnostic workup was initiated. Echocardiography showed normal right ventricular systolic function with no evidence of pulmonary arterial hypertension.[karger.com]
  • The patient was admitted to hospital and given a diagnostic workup. Abdomen ultrasound (USS) showed signs of hepatic steatosis and a poorly defined nodular area in the IV segment. No abnormalities could be detected on chest X-ray.[journalpulmonology.org]
  • Takayasu’s Arteritis - Overview, Presentation, DDx, Workup, Treatment, Medication at by Jefferson R Roberts, MD; Chief Editor: Herbert S Diamond Paul A. Monach, MD, PhD, and Peter A.[medicalfoxx.com]
  • Extensive workup ruled out any ongoing infectious disease.[thoracickey.com]
Mediastinal Mass
  • On chest computed tomography, the wall of the right pulmonary artery was thickened and surrounded by infiltrative mediastinal masses; there were no signs of a currently active tuberculosis (TB) infection.[karger.com]
Treponema Pallidum
  • Laboratory tests revealed a high erythrocyte sedimentation rate (ESR, 81 mm/h), a mildly elevated C-reactive protein (15-25 mg/L, normal range Treponema pallidum particle agglutination assay, hepatitis C virus, antineutrophil cytoplasmic antibodies, antinuclear[karger.com]

Treatment

  • The lesions resolved after two months of steroid treatment. This case shows that we should be aware of atypical GCA manifestations.[journalpulmonology.org]
  • High-dose methylprednisolone treatment (2.5 g over 3 days) was initiated, and methotrexate was started with rapid dose escalation to 20 mg weekly, replacing azathioprine.[karger.com]
  • Both patients responded well to corticosteroid treatment. In a review of the literature, only four additional cases associating lung involvement with temporal arteritis were found.[ncbi.nlm.nih.gov]
  • Successful treatment of a patient with Takayasu's arteritis using a humanized anti-interleukin-6 receptor antibody. Arthritis Rheum. 2008;58:1197-200.[scielo.br]
  • At postoperative three months, iloprost treatment was discontinued.[archivesofrheumatology.org]

Prognosis

  • Key Indexing Terms: TAKAYASU ARTERITIS PULMONARY HYPERTENSION PROGNOSIS VASCULITIS Footnotes Supported by the National Natural Science Foundation of China (grant number: 81170285) and the Research Fund for the Doctoral Program of Higher Education of China[jrheum.org]
  • Maksimowicz-McKinnon K, Clark TM, Hoffman GS (2007) Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum 56:1000–1009 PubMed CrossRef Google Scholar 36.[link.springer.com]
  • Several Turkish studies [ 103, 118 – 121 ] have helped defining the clinical presentation, prognosis and treatment of PAA. This complication can either occur at diagnosis or during the course of BD.[thoracickey.com]
  • Prognosis ILD associated with rheumatoid arthritis causes significant morbidity and mortality. Mortality is high in patients who develop ILD and pulmonary hypertension.[patient.info]
  • […] delay of the diagnosis of disease was 10 months after the onset of first symptoms. 2 Severe hypertension, severe functional disability, and evidence of cardiac involvement were good predictors for either death or major event on follow-up, which help the prognosis[scielo.br]

Etiology

  • Diagnoses of "primary pulmonary arteritis" 2,8 or "pulmonary polyarteritis nodosa" 3,6,7,15 were made, the latter term implying a hyperimmune or allergic etiology.[jamanetwork.com]
  • Giant cell arteritis and Takayasu aortitis: morphologic, pathogenetic and etiologic factors. Int J Cardiol 2000;75:21-33. Brugiere O, Mal H, Sleiman C, Groussard O, Mellot F, Fournier M.[archivesofrheumatology.org]
  • The etiology of TA is not clear. A possible relationship between TA and tuberculosis has been suggested. Some studies suggest cross-reaction between Mycobacterium tuberculosis and human heat shock protein.[jneuro.com]
  • The primary systemic vasculitides are a heterogeneous group of syndromes of unknown etiology, which share a clinical response to immunosuppressive therapy ( Table 74-1 ).[mhmedical.com]

Epidemiology

  • Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary[books.google.com]
  • One of the typical epidemiological features of TA is the marked predominance of the disease in women, with a F/M sex-ratio known to vary from 29/1 to 1.2/1 [ 3 ].[thoracickey.com]
  • Epidemiology Rheumatoid arthritis is often associated with pleural disease (20-40%), interstitial pneumonitis (5-10%), nodules (1%), interstitial fibrosis, bronchiolitis obliterans organising pneumonia, and pulmonary vasculitis.[patient.info]
  • Little epidemiologic data are available on the occurrence of interstitial lung diseases (ILDs) in the general population.[lungindia.com]
  • Giant Cell Arteritis Epidemiology and Treatment. Drugs Aging. 1994;4:135-44. Kyle V et al. The clinical and laboratory course of polymyalgia rheumatica/giant cell arteritis after the first two months of treatment. Ann Rheum Dis. 1993;52:847-50.[rarediseases.org]
Sex distribution
Age distribution

Pathophysiology

  • […] chest movement kyphoscolioisis marked obesity neuromuscular diseases Disorders inducing pulmonary arterial constriction metabolic acidosis hypoxemia chronic altitiude sickness major airway obstruction idiopathic alveolar hypoventilation return to top Pathophysiology[sharinginhealth.ca]
  • Gershwin Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment [3] G.S. Hoffman Giant cell arteritis [4] P. Manganelli, P. Fietta, M. Carotti, A. Pesci, F.[journalpulmonology.org]
  • Arnaud L, Kahn J-E, Girszyn N, Piette AM, Bletry O (2006) Takayasu’s arteritis: an update on pathophysiology. Eur J Intern Med 17:241–246 PubMed CrossRef Google Scholar 4. Seko Y (2007) Giant cell and Takayasu’s arteritis.[link.springer.com]
  • Pathophysiology In this disease, vasculities developed in aorta or its branches which followed a lesion development which is described as a panarteritis with intimal proliferation.[medicalfoxx.com]
  • (See Pathophysiology .) Age and female sex are established risk factors for GCA, a genetic component seems likely, and infection may have a role (see Etiology ).[emedicine.medscape.com]

Prevention

  • Prompt diagnosis and treatment is essential to prevent devastating complications, such as blindness, stroke and aortic aneurysm or dissection. Typical symptoms include headache, temporal artery tenderness and jaw claudication.[journalpulmonology.org]
  • The aim of the treatment of takayasu arteritis is to prevention of further narrowing of the arteries by controlling the vasculities.[medicalfoxx.com]
  • The report was short on advice about what to do to prevent VTE in GCA, but the investigators did recommend “adequate monitoring ... for early recognition of this potentially serious complication.”[mdedge.com]
  • […] problem like indigestion or a stomach ulcer , which can be a side effect of taking prednisolone bisphosphonate therapy – to reduce the risk of osteoporosis when taking prednisolone immunosuppressants – to allow steroid medication to be reduced and help prevent[nhs.uk]
  • Preventive measures may include: Bed rest, low salt diet, a small amount of fluids, diuretics. Disclaimer : The above information is educational purpose.[medigoo.com]

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