Respiratory medicine for MRCP Part 1

A 32-year-old woman of Afro-Caribbean origin has painful red nodules on both shins, ankle arthralgia and mild fever. A chest radiograph shows bilateral hilar lymphadenopathy. She is otherwise well with normal lung function and no eye symptoms. What is the most likely diagnosis?

1. Metastatic malignancy Nodal disease from a solid tumour. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes it a consideration.
2. Lymphoma Lymphadenopathy with systemic symptoms. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well raises it.
3. Berylliosis A granulomatous lung disease from occupational exposure. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well redirects it.
4. Sarcoidosis correct Correct. Erythema nodosum, bilateral hilar lymphadenopathy and arthralgia is Löfgren syndrome, an acute presentation of sarcoidosis.
5. Hypersensitivity pneumonitis Granulomatous lung disease from an inhaled antigen. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well points elsewhere.

The point: Sarcoidosis is a multisystem granulomatous disease of unknown cause, characterised by non-caseating granulomas, most commonly affecting the lungs and intrathoracic lymph nodes. It ranges from an incidental finding of bilateral hilar lymphadenopathy on a chest radiograph to progressive pulmonary fibrosis and multi-organ disease. An acute presentation is Löfgren syndrome, the combination of erythema nodosum, bilateral hilar lymphadenopathy and arthralgia, which has an excellent prognosis and usually resolves without treatment. Other features include uveitis, skin lesions such as lupus pernio, hypercalcaemia (from macrophage activation of vitamin D), cardiac and neurological involvement, and a raised serum ACE. The diagnosis is supported by imaging and a raised ACE and confirmed, where treatment is being considered, by biopsy showing non-caseating granulomas, with tuberculosis excluded. Corticosteroids are reserved for organ-threatening disease: significant or progressive pulmonary involvement, cardiac or neurological disease, hypercalcaemia or sight-threatening eye disease. Asymptomatic hilar lymphadenopathy and Löfgren syndrome do not need steroids.

Source: British Thoracic Society — clinical statement on pulmonary sarcoidosis British Thoracic Society · tier 2, specialty society or college

A 32-year-old woman of Afro-Caribbean origin has painful red nodules on both shins, ankle arthralgia and mild fever. A chest radiograph shows bilateral hilar lymphadenopathy. She is otherwise well with normal lung function and no eye symptoms. What is the most appropriate investigation?

1. Chest radiograph correct Correct. The chest radiograph shows the bilateral hilar lymphadenopathy central to the diagnosis.
2. High-resolution CT of the chest Characterises the lung disease. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes it informative.
3. ECG and eye assessment For cardiac and ocular involvement. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes them thorough.
4. Pulmonary function tests Detect restrictive change and reduced transfer factor. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes them useful.
5. Biopsy showing non-caseating granulomas Confirms the diagnosis where needed. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes it decisive.

The point: Sarcoidosis is a multisystem granulomatous disease of unknown cause, characterised by non-caseating granulomas, most commonly affecting the lungs and intrathoracic lymph nodes. It ranges from an incidental finding of bilateral hilar lymphadenopathy on a chest radiograph to progressive pulmonary fibrosis and multi-organ disease. An acute presentation is Löfgren syndrome, the combination of erythema nodosum, bilateral hilar lymphadenopathy and arthralgia, which has an excellent prognosis and usually resolves without treatment. Other features include uveitis, skin lesions such as lupus pernio, hypercalcaemia (from macrophage activation of vitamin D), cardiac and neurological involvement, and a raised serum ACE. The diagnosis is supported by imaging and a raised ACE and confirmed, where treatment is being considered, by biopsy showing non-caseating granulomas, with tuberculosis excluded. Corticosteroids are reserved for organ-threatening disease: significant or progressive pulmonary involvement, cardiac or neurological disease, hypercalcaemia or sight-threatening eye disease. Asymptomatic hilar lymphadenopathy and Löfgren syndrome do not need steroids.

Source: British Thoracic Society — clinical statement on pulmonary sarcoidosis British Thoracic Society · tier 2, specialty society or college

A 32-year-old woman of Afro-Caribbean origin has painful red nodules on both shins, ankle arthralgia and mild fever. A chest radiograph shows bilateral hilar lymphadenopathy. She is otherwise well with normal lung function and no eye symptoms. What is the most appropriate management?

1. Symptomatic treatment for Löfgren syndrome correct Correct. Löfgren syndrome has an excellent prognosis and needs only symptomatic treatment.
2. Monitor lung function and other organs For progression. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes it ongoing.
3. Observation for asymptomatic hilar lymphadenopathy Asymptomatic hilar disease is observed, as it often resolves without steroids, and erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well here points elsewhere.
4. Corticosteroids for organ-threatening disease Significant lung, cardiac, neurological or eye disease and hypercalcaemia. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes it targeted.
5. Steroid-sparing immunosuppression Such as methotrexate for chronic disease. Erythema nodosum with bilateral hilar lymphadenopathy and arthralgia, otherwise well makes it an escalation.

The point: Sarcoidosis is a multisystem granulomatous disease of unknown cause, characterised by non-caseating granulomas, most commonly affecting the lungs and intrathoracic lymph nodes. It ranges from an incidental finding of bilateral hilar lymphadenopathy on a chest radiograph to progressive pulmonary fibrosis and multi-organ disease. An acute presentation is Löfgren syndrome, the combination of erythema nodosum, bilateral hilar lymphadenopathy and arthralgia, which has an excellent prognosis and usually resolves without treatment. Other features include uveitis, skin lesions such as lupus pernio, hypercalcaemia (from macrophage activation of vitamin D), cardiac and neurological involvement, and a raised serum ACE. The diagnosis is supported by imaging and a raised ACE and confirmed, where treatment is being considered, by biopsy showing non-caseating granulomas, with tuberculosis excluded. Corticosteroids are reserved for organ-threatening disease: significant or progressive pulmonary involvement, cardiac or neurological disease, hypercalcaemia or sight-threatening eye disease. Asymptomatic hilar lymphadenopathy and Löfgren syndrome do not need steroids.

Source: British Thoracic Society — clinical statement on pulmonary sarcoidosis British Thoracic Society · tier 2, specialty society or college