In Station 5 you may be asked to assess a patient with bihilar lymphadenopathy on CXR or alternatively a patient who has bruised tender skins
HISTORY (3 minutes)
- Sarcoidosis questions:
- Eye problems e.g. painful red eye/visual disturbance, skin problems-face and legs e.g. bruised tender shins, joint problems
- Resp: cough, SOB, haemoptysis, wheeze
- CVS: chest pain, palps, syncope
- Abdo: pain, renal stones, polyuria, thirst, urine
- Neuro: headache, face droop, swollen face glands, weakness/numbness
- Systemic: fever, weight loss, fatigue, swollen glands, sweats
- Don’t forget to ask timing questions: since when did these problems start, getting worse, constant/coming and going, relieving/exacerbating factors etc.
- TB questions:
- Fever, night sweats, weight loss, anorexia, fatigue, lymph nodes
- Travel history
- TB contacts
- Have you always lived in the UK? Where were you born?
- BCG vaccine history
- Occupation
- HIV risk assessment:
- Do you or any of your partners inject drugs, have you ever had sex with a man (if male)/bisexual man (if female), have you ever had sex with anyone from Africa/another country, have you ever had sex with a sex worker, have you ever been pain money for sex, have you ever had sex with anyone known to have HIV
- Fever, fatigue, malaise, myalgia, rash, sore throat, headache, swollen glands, joint aches, sweats, diarrhoea, ulcers, weight loss, thrush, shingles
- Cancer screening questions (if not already covered in above) e.g. smoking history, haemoptysis, change in cough etc.
- If history of rash which sounds like erythema nodosum also ask about:
- Strep: sore throat?
- Contraceptive pill, antibiotics, sulphonamides (antibiotics, diabetic drugs, diuretics), phenytoin
- IBD questions: change in bowel habit, bloody stools, abdominal pain, ulcers, weight loss
- Behcet’s disease- any mouth/genital ulcers
- Could you be pregnant?
- Travel history
- PMH (ever had cancer), Fx, Dx (thorough drug history), Sx- occupation (birds, farmers, metal, stone, pottery, light bulbs), pets
EXAMINE (3 minutes)
- Hands- joint swelling, pulse
- Neck- LNs
- Face- rash e.g. lupus pernio, eyes (+fundoscopy if time), parotid enlargement, facial nerve assessment, mouth (throat and tonsils if erythema nodosum)
- Chest- heart, lungs (crepitations), maculopapular rash, axillary LNs
- Abdo- feel for hepatosplenomegaly, inguinal LNs
- Legs- rashes e.g. erythema nodosum
- Neuro- power, peripheral neuropathy
ICE+Explanation (2 minutes)
A suggested explanation could be as follows:
“There are lots of causes of enlarged lymph nodes in the chest area but from what you’ve told me I would like to test for a condition called sarcoidosis. Have you heard of it? It’s a condition in which tiny nodules develop at various sites in the body due to inflammation. The lungs and lymph nodes in the chest area are commonly affected. I’d like to do some blood tests and do a tracing of your heart and some breathing tests to see how well your lungs are working. I’ll also test your urine today. I’ll arrange for you to have a scan of your chest. I’ll refer you to one of the respiratory doctors for further assessment and management”
VIVA
Causes of bihilar lymphadenopathy:
- Sarcoidosis
- Infection- e.g. TB, mycoplasma, HIV, histoplasmosis
- Malignancy- e.g. lymphoma, lung cancer, mets
- Pneumoconiosies eg. Silicosis, berylliosis
Causes of erythema nodosum:
- Infection: streptococcus, TB, mycoplasma, leprosy, Yersinia, salmonella, campylobacter, leptospirosis, brucellosis, chlamydia trachomatis, coccidioidomycosis, histoplasmosis, blastomycosis, psittacosis, infectious mononucleosis, hepatitis B, lymphogranuloma venereum
- Drugs: contraceptive pill, penicillin, sulphonamides (antibiotics, diabetic drugs, diuretics), phenytoin
- Sarcoidosis
- Inflammatory Bowel Disease (crohns more often than ulcerative colitis)
- Behcet’s disease
- Hodgkins lymphoma, leukaemia
- Pregnancy
- Whipple disease
- Sweet syndrome
- Idiopathic
Tests for sarcoidosis:
- Obs and urine dip
- Bloods- ESR, CRP, FBC, LFTs, ACE, Calcium, Immunoglobulins, U+E, vitamin D, TFTs
- 24 hour urine for calcium if raised serum calcium
- Respiratory tests: sats and ambulatory oximetry, CXR, spirometry, BAL, bronchoscopy and biopsy (send biopsy for mycobacterial and fungal testing and histology), HRCT
- Cardio tests: ECG, echo, 24 hour Holter, cardiac MRI
- Abdo tests: urine dipstick, USS, CT
- Eye tests: slit lamp examination, visual acuity, fundoscopic examination
- Neuro tests: CT/MRI, LP
- LN biopsy/skin biopsy/peripheral nerve biopsy
Tests for other causes of bihilar lymphadenopathy:
- Mycoplasma serology
- Tests for TB: Sputum AFBs, early morning urine, mycobacterial culture of biopsy specimen
- Tests for fungal infection e.g. urine and serum antigen testing for histoplasmosis, staining of biopsy specimen
- HIV test
- Blood film, LDH
Tests following diagnosis of erythema nodosum- as per sarcoidosis plus:
- ASO titre
- TB tests- early morning urine, sputum for AFBs
- Mycoplasma serology
- Pregnancy test
- Blood film, LDH
Sarcoidosis:
- Multisystem granulomatous disorder
- North Europe F 20-40yo, blacks more commonly than whites, non-smokers
- Lofgren’s syndrome: bihilar lymphadenopathy, erythema nodosum, fever, weight loss, arthralgia, uveitis
- CXR abnormal in 90%
- BHL 2/3 remit. No treatment needed
- BHL+ infiltrates ½ remit. Treatment with steroid
- Infiltrates 1/3 remit. Treatment with steroid
- Fibrosis Treatment with steroid
- ACE sensitivity 60%, specificity 70%. To monitor disease activity and response to steroid treatment. NOT to diagnose.
- Increased Calcium in 10% (macrophages lead to 1 alpha hydoxylation of vitamin D)
- FBC (low lymphocytes, anaemia, thrombocytopenia), U+E (renal impairment)
- Increased IgG
- HRCT-groundglass and beading
- Spirometry- restrictive pattern
- BAL- increased lymphocytes
- Biopsy- non caseating granulomas and wide alveoli septae. No necrosis.
Granulomatous lung diseases other than sarcoidosis:
- Mycobacterial infection
- Fungal infection e.g. histoplasmosis, blastomycosis, pneumocystis jirovecii
- Hypersensitivity pneumonitis
- Pneumoconisosis e.g. chronic beryllium disease
- Drug-induced hypersensitivity
- Pulmonary histiocytic disorders
- Foreign body granulomatosis
- Vascular inflammation e.g. granulomatosis with polyangiitis (wegener’s), eosinophilic granulomatosis with polyangiitis (churg-strauss)
- Bronchocentric granulomatosis (associated with asthma and allergic bronchopulmonary aspergillosis)
- Primary immunodeficiencies e.g. common variable immunodeficiency
- Immune reconstitution inflammatory syndrome (IRIS) after initiation of antiretroviral therapy (reactivation/appearance of pre-existing subclinical sarcoidosis)
Management of sarcoidosis:
- NSAIDS for arthralgia and skin and bedrest.
- Refer respiratory/opthalmology
- Steroids indicated if:
- CXR 2-4 (see above)
- Eye problems
- Raised Calcium
- Neurological involvement
- Cardiac involvement
- 40mg prednisolone for 4-6 weeks then reduce dose (20% respond). Remember bone and gastroprotection. Do baseline DEXA scan.
- Monitor with ESR and ACE
- Steroid sparing agents = methotrexate, hydroxychloroquine, ciclosporin, cyclophosphamide, infliximab
- Surgery- lung transplant, pacemaker
Erythema Nodosum
This is a septal panniculitis. There is inflammation in subcutaneous septa of fat. It affects young females predominantly. It causes raised purple/red tender plaques on the shins
Management of Erythema Nodosum:
Treat the cause e.g. If on contraceptive pill stop it. Rest. Elevate leg. NSAIDs. Usually resolves in 6 weeks without scarring
Written by Dr Sarah Kennedy
Resources used to write this document include those listed in the references section of this webpage and also:
https://patient.info/doctor/sarcoidosis-pro