A 55 year old man has numbness in hands and feet. His medical history is significant for a asthma associated with nasal polyps and facial pain. Eosinophilia was shown on his last blood test. A pulmonary CT scan shows ground-glass inflammation. What is the SINGLE most likely diagnosis?
A. Churg-Strauss Syndrome
B. Temporal arteritis
C. Polyarteritis Nodosa
D. Wegener granulomatosis
E. Inflammatory myopathies
The diagnosis here is Churg-Strauss Syndrome. The incidence of Churg-Strauss Syndrome is rare thus it is also rarely asked in PLAB. However, one might see this topic every now and then.
Churg-Strauss Syndrome
A rare diffuse vasculitic disease affecting coronary, pulmonary, cerebral, abdominal visceral and skin circulations. The vasculitis affects small- and medium-sized arteries
and veins and is associated with asthma. The cardinal manifestations of Churg-Strauss syndrome are asthma, eosinophilia, and lung involvement.
The incidence of Churg-Strauss Syndrome is rare thus it is also rarely asked in PLAB. However, one might see this topic every now and then.
The incidence of Churg-Strauss Syndrome is rare thus it is also rarely asked in PLAB. However, one might see this topic every now and then.
The American College of Rheumatology has identified six criteria for the diagnosis of Churg-Strauss Syndrome - Asthma (wheezing, expiratory rhonchi) - Eosinophilia of more than 10% in peripheral blood - Paranasal sinusitis - Pulmonary infiltrates (may be transient) - Histological confirmation of vasculitis with extravascular eosinophils - Mononeuritis multiplex or polyneuropathy
The presence of four out of six of these features has a high specificity and sensitivity for the diagnosis of Churg-Strauss Syndrome
Presentation The most prominent symptoms and signs include: - Pulmonary: asthma - Upper respiratory tract: allergic rhinitis, paranasal sinusitis, nasal polyposis. - Cardiac involvement is common. This includes heart failure, myocarditis and myocardial infarction - Skin: purpura, skin nodules - Renal: glomerulonephritis - Peripheral neuropathy: mononeuritis multiplex is the most frequent form
Investigations - Antineutrophil cytoplasmic antibodies (ANCAs): 30-40% of patients are perinuclear staining (p-ANCA) positive (antimyeloperoxidase antibodies) - Eosinophilia and anaemia on the FBC - Elevated ESR and CRP - Elevated serum creatinine - Increased serum IgE levels - CXR: pulmonary opacities, transient pulmonary infiltrates, and bilateral multifocal consolidation - High-resolution CT: Ground-glass attenuation Biopsy: small necrotising granulomas, as well as necrotising vasculitis (found especially in the lung)