Australian Doctor 19th April issue | Page 32

32 HOW TO TREAT : SARCOIDOSIS

32 HOW TO TREAT : SARCOIDOSIS

19 APRIL 2024 ausdoc . com . au
Asymptomatic / tolerable symptoms and no endorgan injury
Observation
Figure 7 . Management algorithm for pulmonary sarcoidosis .
Cardiology consult for consideration of pacemaker / implantable defibrillator
Pulmonary sarcoidosis
Good clinical response and successful taper of glucocorticoids
Cardiac sarcoidosis
And
Glucocorticoids
Glucocorticoid side effects or progression of disease despite therapy
End-organ injury or persistent poorly tolerated symptoms
Glucocorticoids
Glucocorticoid side effects or progression of disease despite therapy
Methotrexate ( azathioprine and mycophenolate may be considered )
Persistent / progressive disease or reintroduction of glucocorticoids
Infliximab ( anti-TNFα )
The authors recommend specialist multidisciplinary team assessment for direction of therapy at this stage
Based on Baughman RP et al 2021 20 Based on Baughman RP et al 2021 20
PAGE 30 male of African descent , presents to a chest clinic as a visa requirement . He has a positive screening chest X-ray that demonstrates bilaterally enlarged hilar nodes . He reports exertional breathlessness that is uncharacteristic for his age and body habitus . Interferon-gamma release assay is positive , while three early-morning sputa samples for mycobacteria are negative . CT chest ( see figure 13 ) demonstrates parenchymal nodular changes and mediastinal lymphadenopathy . He undergoes bronchoscopy and EBUS , which demonstrate non-necrotising granuloma . Bronchoscopic cultures do not demonstrate growth of mycobacterium .
He subsequently returns to the clinic with a new dry cough and bilateral uveitis ( see figure 14 ). Auscultation of his chest reveals bilateral fine basal inspiratory crepitations . Extrapulmonary examination findings and histopathology make the clinical suspicion of sarcoidosis high and the likelihood of active TB low ; oral prednisolone is started .
On return six weeks later , his uveitis has resolved , and his dyspnoea is much improved . This case reflects the clinical conundrum of
Mycobacterium tuberculosis screening and sarcoidosis . While not common in Australia , the worldwide incidence of both TB and severe sarcoidosis is higher . Given the high pre-test probability of active TB , hesitation to commence glucocorticoids is reasonable ; however , the combination of clinical findings , negative culture for TB and capacity for close and regular follow-up makes steroid pharmacotherapy the most appropriate way forward .
Case study four
Steven , a 53-year-old Caucasian male , presents to hospital and is incidentally found to have extensive mediastinal and hilar lymphadenopathy and upper lobe parenchymal nodules . He only recently had an automatic implantable cardioverter defibrillator inserted for heat block . An FDG – PET scan demonstrates intense uptake in the pulmonary nodules , bilateral hilar and mediastinal lymph nodes , cervical lymph nodes , liver , inguinal nodes , multiple bilateral ribs , vertebrae and pelvis . In addition , intense pericardial and myocardial FDG uptake is noted ( see figure 15 ). Interestingly , he is mostly asymptomatic PAGE 34
Methotrexate ( azathioprine and mycophenolate may be considered )
Good clinical response and successful taper of glucocorticoids
Figure 8 . Management algorithm for cardiac sarcoidosis .
Evidence for steroid-sparing agents is weaker in cardiac sarcoidosis than pulmonary sarcoidosis .
Persistent / progressive disease or reintroduction of glucocorticoids
Infliximab ( anti-TNFα ) or cyclophosphamide
Observation
Treatment of affected organs as appropriate
No effect from treatment of specific organ disease
Sarcoidosis-associated fatigue
Exercise training program
Other cause of fatigue identified
Treatment as appropriate
Based on Baughman RP et al 2021 20
Figure 10 . CT pulmonary angiogram demonstrating mediastinal and hilar lymphadenopathy consistent with stage I sarcoidosis . Arrows indicate mediastinal ( top ) and hilar nodes ( bottom ).
Persistent fatigue
Armodafinil
Persistent fatigue
Trial low-dose glucocorticoids or steroidsparing agent
Figure 9 . Management algorithm for sarcoidosisassociated fatigue .
Figure 11 . Chest X-ray showing subtle upper lobe – predominant nodules in bilateral lung fields .