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 Node disease spreading from a solid cancer. But tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well does not point to it.
2. Lymphoma Swollen lymph nodes with body-wide symptoms. It does not fit tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well.
3. Berylliosis A granuloma-forming lung disease from exposure to beryllium at work. That does not match tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well.
4. Sarcoidosis correct Correct. Tender red shin lumps, swollen lymph nodes on both sides near the lung roots, and joint aches is Löfgren syndrome, a sudden presentation of sarcoidosis.
5. Hypersensitivity pneumonitis Lung disease with granulomas from breathing in a trigger substance. Here, tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well points elsewhere.

The point: Sarcoidosis is a disease of unknown cause that affects several organs and makes clumps of inflammatory cells called non-caseating granulomas (granulomas without dead tissue in the middle, which is what separates it from tuberculosis). It most often affects the lungs and the lymph nodes in the chest. It can range from an incidental finding of swollen lymph nodes on both sides near the lung roots (bilateral hilar lymphadenopathy) on a chest x-ray, to scarring of the lungs (pulmonary fibrosis) that gets worse over time and disease affecting several organs. A sudden presentation is Löfgren syndrome: tender red lumps on the shins (erythema nodosum) together with swollen lymph nodes on both sides near the lung roots and joint aches (arthralgia). It has a very good outlook and usually gets better on its own. Other features include eye inflammation (uveitis), skin patches such as lupus pernio, high blood calcium (from immune cells activating vitamin D), heart and nerve involvement, and a raised blood test called serum ACE. The diagnosis is supported by scans and a raised ACE, and confirmed, when treatment is being considered, by a biopsy showing non-caseating granulomas, once tuberculosis has been ruled out. Steroid tablets are kept for disease that threatens an organ: serious or worsening lung disease, heart or nerve disease, high calcium, or eye disease that threatens sight. Lymph node swelling with no symptoms, 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. Treat hypercalcaemia From immune cells activating vitamin D. But tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well does not point to it.
2. Monitor lung function and other organs In case it gets worse. Here, tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well points elsewhere.
3. Steroid-sparing immunosuppression Such as methotrexate for disease that lasts a long time. That does not match tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well.
4. Corticosteroids for organ-threatening disease Serious lung, heart, nerve or eye disease, and high calcium. It does not fit tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well.
5. Symptomatic treatment for Löfgren syndrome correct Correct. Löfgren syndrome has a very good outlook and needs only treatment for the symptoms.

The point: Sarcoidosis is a disease of unknown cause that affects several organs and makes clumps of inflammatory cells called non-caseating granulomas (granulomas without dead tissue in the middle, which is what separates it from tuberculosis). It most often affects the lungs and the lymph nodes in the chest. It can range from an incidental finding of swollen lymph nodes on both sides near the lung roots (bilateral hilar lymphadenopathy) on a chest x-ray, to scarring of the lungs (pulmonary fibrosis) that gets worse over time and disease affecting several organs. A sudden presentation is Löfgren syndrome: tender red lumps on the shins (erythema nodosum) together with swollen lymph nodes on both sides near the lung roots and joint aches (arthralgia). It has a very good outlook and usually gets better on its own. Other features include eye inflammation (uveitis), skin patches such as lupus pernio, high blood calcium (from immune cells activating vitamin D), heart and nerve involvement, and a raised blood test called serum ACE. The diagnosis is supported by scans and a raised ACE, and confirmed, when treatment is being considered, by a biopsy showing non-caseating granulomas, once tuberculosis has been ruled out. Steroid tablets are kept for disease that threatens an organ: serious or worsening lung disease, heart or nerve disease, high calcium, or eye disease that threatens sight. Lymph node swelling with no symptoms, 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 definitive treatment?

1. Treat hypercalcaemia From immune cells activating vitamin D. That does not match tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well.
2. Observation for asymptomatic hilar lymphadenopathy correct Correct. Lymph node swelling with no symptoms is watched, as it often gets better without steroids.
3. Steroid-sparing immunosuppression Such as methotrexate for disease that lasts a long time. But tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well does not point to it.
4. Corticosteroids for organ-threatening disease Serious lung, heart, nerve or eye disease, and high calcium. It does not fit tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well.
5. Monitor lung function and other organs In case it gets worse. Here, tender red shin lumps with swollen lymph nodes on both sides and joint aches, otherwise well points elsewhere.

The point: Sarcoidosis is a disease of unknown cause that affects several organs and makes clumps of inflammatory cells called non-caseating granulomas (granulomas without dead tissue in the middle, which is what separates it from tuberculosis). It most often affects the lungs and the lymph nodes in the chest. It can range from an incidental finding of swollen lymph nodes on both sides near the lung roots (bilateral hilar lymphadenopathy) on a chest x-ray, to scarring of the lungs (pulmonary fibrosis) that gets worse over time and disease affecting several organs. A sudden presentation is Löfgren syndrome: tender red lumps on the shins (erythema nodosum) together with swollen lymph nodes on both sides near the lung roots and joint aches (arthralgia). It has a very good outlook and usually gets better on its own. Other features include eye inflammation (uveitis), skin patches such as lupus pernio, high blood calcium (from immune cells activating vitamin D), heart and nerve involvement, and a raised blood test called serum ACE. The diagnosis is supported by scans and a raised ACE, and confirmed, when treatment is being considered, by a biopsy showing non-caseating granulomas, once tuberculosis has been ruled out. Steroid tablets are kept for disease that threatens an organ: serious or worsening lung disease, heart or nerve disease, high calcium, or eye disease that threatens sight. Lymph node swelling with no symptoms, 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