Respiratory for AMC MCQ

A 32 year old woman presents with fever, painful red nodules on her shins, and painful, swollen ankles. A chest radiograph shows bilateral hilar lymphadenopathy. She is otherwise well with a normal calcium. Her symptoms are self-limiting over a few weeks with simple analgesia. What is the most likely diagnosis?

1. Tuberculosis Lymphadenopathy with weight loss and caseating granulomas, the key mimic. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it worth excluding.
2. Hypersensitivity pneumonitis Interstitial lung disease from an inhaled antigen. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves points elsewhere.
3. Sarcoidosis correct Correct. Bilateral hilar lymphadenopathy with erythema nodosum, arthralgia and fever is Lofgren syndrome, a form of sarcoidosis.
4. Berylliosis A granulomatous lung disease from occupational exposure. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes the history matter.
5. Metastatic malignancy Mediastinal nodes from a distant cancer. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it worth considering.

The point: Sarcoidosis is a multisystem granulomatous disease of unknown cause, commonest in young and middle-aged adults. The lungs are involved in most cases, and the classic chest radiograph shows bilateral hilar lymphadenopathy, with or without pulmonary infiltrates. Lofgren syndrome is an acute, often self-limiting presentation with bilateral hilar lymphadenopathy, erythema nodosum, fever and arthralgia. Other features are anterior or posterior uveitis, skin lesions including lupus pernio, and hypercalcaemia from granulomatous activation of vitamin D. The serum angiotensin-converting enzyme may be raised but is not diagnostic. The diagnosis rests on a compatible picture with non-caseating granulomas on biopsy, and the main mimic to exclude is tuberculosis. Many cases, particularly Lofgren syndrome, resolve without treatment, and corticosteroids are reserved for organ-threatening disease such as significant lung, cardiac, neurological or eye involvement, or symptomatic hypercalcaemia. Cardiac and neurological sarcoidosis, though uncommon, are the dangerous forms.

Source: Therapeutic Guidelines (Australia) — Respiratory: sarcoidosis Therapeutic Guidelines (Australia) · tier 3, national formulary

A 32 year old woman presents with fever, painful red nodules on her shins, and painful, swollen ankles. A chest radiograph shows bilateral hilar lymphadenopathy. She is otherwise well with a normal calcium. Her symptoms are self-limiting over a few weeks with simple analgesia. What is the most appropriate initial investigation?

1. Lung function tests Assess restrictive change and gas transfer. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves indicates it.
2. Exclusion of tuberculosis The main mimic, excluded before treating. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it necessary.
3. Serum angiotensin-converting enzyme Often raised but not diagnostic. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it supportive.
4. Chest radiograph correct Correct. It showed the bilateral hilar lymphadenopathy that is central to the diagnosis.
5. Assessment for cardiac and neurological involvement The dangerous forms. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it important.

The point: Sarcoidosis is a multisystem granulomatous disease of unknown cause, commonest in young and middle-aged adults. The lungs are involved in most cases, and the classic chest radiograph shows bilateral hilar lymphadenopathy, with or without pulmonary infiltrates. Lofgren syndrome is an acute, often self-limiting presentation with bilateral hilar lymphadenopathy, erythema nodosum, fever and arthralgia. Other features are anterior or posterior uveitis, skin lesions including lupus pernio, and hypercalcaemia from granulomatous activation of vitamin D. The serum angiotensin-converting enzyme may be raised but is not diagnostic. The diagnosis rests on a compatible picture with non-caseating granulomas on biopsy, and the main mimic to exclude is tuberculosis. Many cases, particularly Lofgren syndrome, resolve without treatment, and corticosteroids are reserved for organ-threatening disease such as significant lung, cardiac, neurological or eye involvement, or symptomatic hypercalcaemia. Cardiac and neurological sarcoidosis, though uncommon, are the dangerous forms.

Source: Therapeutic Guidelines (Australia) — Respiratory: sarcoidosis Therapeutic Guidelines (Australia) · tier 3, national formulary

A 32 year old woman presents with fever, painful red nodules on her shins, and painful, swollen ankles. A chest radiograph shows bilateral hilar lymphadenopathy. She is otherwise well with a normal calcium. Her symptoms are self-limiting over a few weeks with simple analgesia. What is the most appropriate next step in management?

1. Treat symptomatic hypercalcaemia Steroids and avoiding vitamin D and sunlight. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it necessary.
2. Steroid-sparing agents for chronic disease Where long-term treatment is needed. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it the escalation.
3. Observation for self-limiting disease correct Correct. Lofgren syndrome is usually self-limiting, so observation with symptomatic treatment is appropriate.
4. Ophthalmology referral for uveitis For eye involvement. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it appropriate.
5. Symptomatic treatment of erythema nodosum and arthralgia For the acute presentation. Hilar lymphadenopathy with erythema nodosum and arthralgia that self-resolves makes it supportive.

The point: Sarcoidosis is a multisystem granulomatous disease of unknown cause, commonest in young and middle-aged adults. The lungs are involved in most cases, and the classic chest radiograph shows bilateral hilar lymphadenopathy, with or without pulmonary infiltrates. Lofgren syndrome is an acute, often self-limiting presentation with bilateral hilar lymphadenopathy, erythema nodosum, fever and arthralgia. Other features are anterior or posterior uveitis, skin lesions including lupus pernio, and hypercalcaemia from granulomatous activation of vitamin D. The serum angiotensin-converting enzyme may be raised but is not diagnostic. The diagnosis rests on a compatible picture with non-caseating granulomas on biopsy, and the main mimic to exclude is tuberculosis. Many cases, particularly Lofgren syndrome, resolve without treatment, and corticosteroids are reserved for organ-threatening disease such as significant lung, cardiac, neurological or eye involvement, or symptomatic hypercalcaemia. Cardiac and neurological sarcoidosis, though uncommon, are the dangerous forms.

Source: Therapeutic Guidelines (Australia) — Respiratory: sarcoidosis Therapeutic Guidelines (Australia) · tier 3, national formulary