In our #AMreport today at Women’s College Hospital, we reviewed a case of focal lymphadenopathy which turned out to be sarcoidosis with pulmonary manifestations. Below are the take home points on head and neck lymphadenopathy, biopsies and pulmonary sarcoid.
Take Home Points: Head & Neck Lymphadenopathy
- Biopsy of a localized lymph node should be expedited in patients where a node has persisted for more than four weeks and is associated with systemic features concerning for malignancy.
Core biopsy is useful in terms of cost-effectiveness as it gives you details about microarchitecture and does not have as high a false positive rate as FNA biopsy. The microarchitecture is important for histologic classification and plays an important role in determining treatment regimens particularly for lymphoma.
Take Home Points: Pulmonary Sarcoidosis
Chest X-Ray Classification of Pulmonary Sarcoid
Stage I: Bilateral hilar adenopathy, often with right paratracheal node enlargement
~50% of patients
Stage II: Bilateral hilar adenopathy and reticular opacities (upper lung zones usually).
~25% of patients
Stage III: Reticular opacities (upper lung zones) with shrinking hilar nodes.
Stage IV: Reticular opacities with evidence of volume loss, predominantly in the upper lung zones; can have conglomerated masses with traction bronchiectasis, as well as calcification alwong with cystic formation.
Apart from biopsy BAL washings are useful in determining diagnosis.
- Can be used as an adjunctive measure by showing reduced number of CD8 cells, an increased CD4:CD8 (>4:1) ratio and increased lymphocytes (>16%)
- ~100% PPV for sarcoidosis if: CD4:CD8 >4:1, lymphocyte % >16, biopsy showing non-caseating granulomas.
- Most patients with pulmonary sarcoidosis do not require treatment.
- As a general rule thumb if organ function is threatened then implement glucocorticoid therapy.
Indications for therapy include:
1.Deteriorating lung function, as assessed by serial testing at three to six month intervals showing:
- A fall in total lung capacity (TLC) of 10 percent or more
- A fall in forced vital capacity (FVC) of 15 percent or more
- A decrease in diffusing capacity (DLCO) of 20 percent or more; or worsened gas exchange at rest or with exercise
3.Progressive radiographic changes, with fibrosis or honeycombing, or development of signs of pulmonary hypertension
Treatment usually entails:
- Prednisone 0.3-0.6 mg/kg (typically 20-40 mg day)
- Usually treated for 3 months and then response to therapy evaluated.
- If no response after 3 months then alternative therapy with other immunosuppressants should be considered (methotrexate/biologics).
- If positive response after 3 months then steroids are tapered to a dose of 5-15 mg daily and treated for a further 9-12 months.
- Treatment should be coordinated in consultation and close follow-up with a respirologist/rheumatologist.
Excellent review article on sarcoidosis here: http://www.nejm.org/doi/full/10.1056/NEJMra071714