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]

  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]

  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]

  Symptoms: Some of the early signs of cor pulmonale include constant cough, difficulty breathing, fatigue and weakness. [medigoo.com]

  For this reason the oxygenated blood circulation is less in the organs and tissues and following symptoms arise: During any type of physical activity, the upper or lowers limbs become easily fatigue and pain symptoms started. [medicalfoxx.com]

• Congestive Heart Failure

  Congestive heart failure and chronic kidney disease were more prevalent in the GCA population ( P ≤0.001), while coronary artery disease and chronic obstructive pulmonary disease were more frequent in those without GCA ( P In-hospital mortality was 4.1% [medpagetoday.com]

  The frequent findings with takayasu arteritis are cardiac disorder, including congestive heart failure, but cardiomyopathy, myocarditis, and pericarditis are also reported with takayasu arteritis. [medicalfoxx.com]

  Major causes of death are congestive heart failure and cerebrovascular complications [ 1, 2, 33 ]. [bmcpulmmed.biomedcentral.com]

  Elevated PTX3 levels have been reported in many types of cardiovascular disease, including acute coronary syndrome [12], [13], congestive heart failure [18], and heart failure with normal ejection fraction [19]. [journals.plos.org]

  Park et al. [ 96 ] underlined that major prognostic factors in TA were presence of valvular heart disease, cerebrovascular accidents, congestive heart failure, ischemic heat disease, retinopathy and renovascular hypertension [ 96 ]. [thoracickey.com]

• Anemia

  Symptoms may include low-grade fever, muscle pain (myalgia), low levels of circulating red blood cells (mild anemia), and/or severe stiffness, especially in the morning or after a period of inactivity (gel phenomenon). [rarediseases.org]

  Common findings include anemia of chronic disease, elevated platelet levels, occasionally elevated WBC counts, and elevated ESR and C-reactive protein. [msdmanuals.com]

  Laboratory findings showed normal white blood cells with predominant lymphocytosis, increased erythrocytes sedimentation rate (50 mm), C-reactive protein 63 g/l (normal < 6 g/l) and anemia. [jneuro.com]

  Leukocytosis, anemia of chronic disease, a high erythrocyte sedimentation rate (ESR) and an elevated C-reactive protein level are commonly found in most types of vasculitis. [aafp.org]

• Hypoxemia

  Our data suggested that 9 patients with PH due to PA presented with hypoxemia. [jrheum.org]

  […] granulomatosis) drug-, toxin-, or radiation-induced vascular obstruction extensive tumour microembolism Disorders affecting chest movement kyphoscolioisis marked obesity neuromuscular diseases Disorders inducing pulmonary arterial constriction metabolic acidosis hypoxemia [sharinginhealth.ca]

  Contrast echocardiography Minor right to left shunting rarely produces hypoxemia. Doppler echocardiography This is too unreliable for following serial measurements to monitor therapy. [slideplayer.com]

• 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]

• Heart Disease

  Hypertension due to Lung Disease and/or Hypoxia, Pulmonary Arterial Hypertension Associated with Congenital Heart Disease, World Health Organization Group 5 Pulmonary Hypertension, and more! [books.google.com]

  ICD-10-CM Codes › I00-I99 Diseases of the circulatory system › I26-I28 Pulmonary heart disease and diseases of pulmonary circulation › I27- Other pulmonary heart diseases › Pulmonary heart disease, unspecified 2016 2017 2018 2019 Billable/Specific Code [icd10data.com]

  (a1) 1 Congenital Heart Disease Unit, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson, France (a2) 2 Thorax Institute, Centre Hospitalo-Universitaire de Nantes, Nantes, France (a3) 3 Congenital Heart Disease Unit, Centre Hospitalo-Universitaire [cambridge.org]

  Among the 48 patients with TA who had PH, 36 (75.0%) had PA, and left heart disease (LHD) was present in 12 (25.0%). [jrheum.org]

• Hepatomegaly

  Secondary compression of the left ventricular chamber or tricuspid regurgitation can follow fibrous thickening of the valve. return to top Signs and Symptoms history physical exam Physical Exam Signs of right-sided hearted failure include: increased JVP hepatomegaly [sharinginhealth.ca]

  In the cardiovascular examination there was jugular venous distension, hepatomegaly, hepatojugular reflux, ascites and oedema of lower limbs. [err.ersjournals.com]

  Per abdomen examination revealed soft, tender hepatomegaly. A provisional diagnosis of interstitial lung disease with cor pulmonale was kept. [lungindia.com]

• Blurred Vision

  Case report A 74-year-old man was observed at the emergency department for low grade fever (38 °C), bilateral headaches and blurred vision of the left eye, which had started 10 days earlier. [journalpulmonology.org]

/takayasus-arteritis/ by David Hellmann, M.D. http://www.medicinenet.com/takayasu_disease/article.htm http://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/basics/symptoms/con-20028085 Takayasu’s Arteritis -> Overview, Presentation, DDx, Workup [medicalfoxx.com]

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]

Extensive workup ruled out any ongoing infectious disease. [thoracickey.com]

All spiral CT images were systematically read during the course of clinical workup by senior radiologists. [jrheum.org]

• Chlamydia

  A possible association between Toll-like receptor 4 gene polymorphism and susceptibility to biopsy-proven GCA has been found. [23] Epidemiological observations, reports, and studies using DNA detection techniques have implicated Chlamydia pneumoniae, [emedicine.medscape.com]

Patients treated with Actemra who develop a serious infection should stop that treatment until the infection is controlled. Live vaccines should be avoided during treatment with Actemra. [fda.gov]

For the vast majority of people with vasculitis, treatment is very effective. [resources.lupus.org]

Standard Therapies Treatment The treatment of giant cell arteritis should start when the diagnosis is suspected. Prompt treatment may help to avoid serious complications such as blindness and coronary blockage (occlusion). [rarediseases.org]

While treatment for RA has improved, treatment for lung disease has not. The goal of treatment is early stage intervention to slow the progression of the disease and improve quality of life. [healthline.com]

The lesions resolved after two months of steroid treatment. This case shows that we should be aware of atypical GCA manifestations. [journalpulmonology.org]

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]

Prognosis ILD associated with rheumatoid arthritis causes significant morbidity and mortality. Mortality is high in patients who develop ILD and pulmonary hypertension. [patient.info]

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]

Prognosis Complete cure of takayasu arteritis is not possible, but right diagnosis and early initiation of treatment can prevent the further complication and provide quality of life to the patient. [medicalfoxx.com]

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]

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]

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]

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]

[…] 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]

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]

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]

(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]

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]

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]