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Clinical manifestationsPeripheral Arterial Disease (PAD) manifests through a range of symptoms, from the characteristic intermittent claudication to atypical presentations and severe complications in advanced stages. Intermittent claudication, a hallmark symptom of PAD, presents as exercise-induced muscle pain that typically resolves within minutes of rest. This pain is reproducible and stems from inadequate blood flow, leading to the accumulation of lactic acid produced during anaerobic...
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Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
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Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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Esophageal varices often manifest as gastrointestinal bleeding episodes, presenting symptoms like hematemesis (vomiting of blood), hematochezia (passing fresh blood via the rectum), and melena (black, tarry stools). Other signs can include weight loss, anorexia, abdominal discomfort, jaundice, pruritus, altered mental status, and muscle cramps.
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Polyangiitis overlap syndrome: a rare clinical entity.

Lorenza Bruno1, Martina Mandarano2, Guido Bellezza2

  • 1Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, 06129, Perugia, PG, Italy.

Rheumatology International
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PubMed
Summary

Polyangiitis overlap syndrome, a rare condition, presents with overlapping vasculitis features. This case highlights a successful cyclophosphamide treatment for a patient with anti-PR3 antibodies and lung involvement.

Keywords:
ANCAAnti-PR3EGPAEosinophiliaEosinophilic granulomatosis with polyangiitisGranulomatosis with polyangiitisVasculitis

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Area of Science:

  • Rheumatology
  • Immunology

Background:

  • Polyangiitis overlap syndrome (POS) is a rare vasculitic entity, often involving eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA).
  • POS typically presents with c-ANCA and anti-proteinase (PR)-3 positivity, exhibiting diverse clinical features including vasculitis, eosinophilia, and tissue eosinophilic infiltrates.

Observation:

  • A 66-year-old woman presented with nasal obstruction, external nose deformity, sensorineural hearing loss, peripheral blood eosinophilia, high-titer anti-PR3 antibodies, and lung involvement.
  • Nasal septum biopsies revealed an inflammatory infiltrate with an eosinophilic component.
  • Lung histopathology showed necrotizing granulomas with neutrophil and eosinophil-rich inflammatory infiltrates.

Findings:

  • The patient was diagnosed with polyangiitis overlap syndrome based on clinical and histopathological findings.
  • The patient demonstrated a favorable response to immunosuppressive therapy, specifically cyclophosphamide.

Implications:

  • Recognizing POS is crucial due to its distinct clinical phenotype and therapeutic responses within ANCA-associated vasculitides.
  • This case underscores the importance of considering POS in patients with overlapping vasculitis features and anti-PR3 positivity.