34 HOW TO TREAT : SARCOIDOSIS
34 HOW TO TREAT : SARCOIDOSIS
19 APRIL 2024 ausdoc . com . au
Figure 14 . Bilateral uveitis secondary to sarcoidosis .
Figure 12 . CT pulmonary angiogram demonstrating bilateral upper lobe – predominant reticular pattern with peri-lymphatic micronodules .
Figure 15 . FDG – PET scan images demonstrating diffuse myocardial uptake with predilection for the posteriolateral and septal walls . Fused CT / PET images ( left ); maximum intensity projection PET images ( right ).
Figure 16 . Cardiac MRI demonstrating late gadolinium enhancement in cardiac sarcoidosis ( red arrow ). Patchy subepicardial distribution may indicate irreversible damage and is a risk factor for cardiac arrythmia .
Figure 13 . CT chest demonstrating bilateral basal , predominantly nodular , changes .
How to Treat Quiz .
1 . Which THREE statements regarding sarcoidosis are correct ? a More than 90 % of presentations are pulmonary . b It is a multisystem inflammatory disorder characterised by necrotising granulomatous inflammation . c Multiple organ systems may be implicated in a patient ’ s sarcoid phenotype . d Sarcoidosis in the Australian context is often mild and can be managed without steroids or other immunomodulatory agents .
2 . Which TWO statements regarding the epidemiology of sarcoidosis are correct ? a Sarcoidosis is equally common in men and women . b The reported incidence in Australia may be a significant underestimation . c Sarcoidosis is always a sporadic disease . d The peak incidence appears between the ages of 20 and 39 .
3 . Which THREE are possible presentations of sarcoidosis in the lung ? a Recurrent pneumonia . b Intrathoracic lymphadenopathy . c Pulmonary fibrosis . d Chronic cough .
4 . Which ONE statement regarding hypercalcaemia in sarcoidosis is incorrect ? a It occurs in about 7-18 % of patients with sarcoidosis . b It is safe to assume that hypercalcaemia in a patient with known sarcoidosis is a feature of the condition . c Symptoms include fatigue , weakness , muscle and bone pain , constipation and kidney stones . d The management of hypercalcaemia in sarcoidosis typically involves a combination of medications , hydration and lifestyle changes .
5 . Which THREE criteria are required to make a diagnosis of sarcoidosis ? a A compatible clinical presentation . b The presence of non-necrotising granuloma on biopsy . c Clinical symptoms present for at least six months . d Exclusion of other causes of granulomatous disease .
PAGE 32 aside from a dry cough . Comprehensive lung function testing is within normal limits .
The initial thought is a lymphoproliferative process , but bone marrow shows no abnormal cell population ,
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6 . Which TWO statements regarding sarcoidosis are correct ? a Typical imaging can range from a normal chest X-ray to bilateral hilar node enlargement or gross fibrotic changes . b Those with only hilar or mediastinal lymphadenopathy ( Scadding stage I ) have a 60 - 90 % spontaneous remission rate . c Chest CT is the initial screening modality . d All incidentally discovered mediastinal / hilar lymphadenopathy requires biopsy .
7 . Which THREE statements regarding the investigation of sarcoidosis are correct ? a EBUS – TBNA has a vastly greater diagnostic yield when compared with traditional TBNA . b FBC , EUC , calcium and LFTs are initial investigations . c Order a specific urine calcium excretion test . d Serum ACE is key in the diagnosis of sarcoidosis .
8 . Which TWO statements regarding the management of
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SARCOIDOSIS
and an excision mediastinal lymph node biopsy demonstrates non-caseating granulomata . He is observed over 18 months , and a repeat PET scan shows increased uptake in the pericardium and myocardium . A
sarcoidosis are correct ? a Most Australian patients require long-term steroids or immunosuppressive therapies . b Screening for steroid-induced diabetes , hypertension and osteoporosis is crucial when patients are on long-term steroids . c Consider steroid-sparing agents when there is disease progression despite glucocorticoid therapy or if glucocorticoid toxicity becomes unacceptable . d Monoclonal antibodies are the initial second-line agent of choice in most patients .
9 . Which THREE are appropriate in the management of cardiac sarcoidosis ? a Annual surveillance only . b Implanted pacemaker with or without a defibrillator . c Glucocorticoids . d Steroid-sparing agents .
10 . Which TWO statements regarding sarcoid-associated fatigue are correct ? a It is experienced by up to 90 % of those with sarcoidosis . b Initial therapy is anti-inflammatory therapies . c Exercise training programs can be ceased when the fatigue starts to settle . d It is important to exclude other differential diagnoses of fatigue . subsequent MRI scan demonstrates patchy regions of mid-myocardial and subepicardial late gadolinium enhancement ( see figure 16 ). After discussion at an interstitial lung disease multidisciplinary team meeting , a combination of prednisone and methotrexate is started . After 18 months , a repeat PET scan shows near-complete resolution of the cardiac , pulmonary , nodal and skeletal uptake , indicating a disease-modifying response to the immunomodulatory therapy . Steven remains asymptomatic ; the prednisone is weaned over the next 18 months and the methotrexate after five years . Ongoing surveillance is planned .
CONCLUSION
SARCOIDOSIS is a chronic multiorgan disease that predominantly presents as an incidental finding or with minimal symptoms in Australian Caucasian populations . However , manifestation in organ systems — such as the heart , nervous system and eyes — can be life-threatening or severely debilitating . Corticosteroid therapy is a well-trialled effective therapy and should provide relief for most symptomatic patients .
Diagnosis can be challenging , but the advent of safe biopsy techniques with high diagnostic yields via EBUS are helping overcome this issue .
The role of GPs is multifaceted , ranging from identification of those who require further diagnostic investigation for sarcoidosis to the longerterm monitoring of treatment side effects . Patients , respiratory physicians and other specialists alike rely on GPs to provide chronic care management and to identify disease relapse or progression so it may be managed efficiently to limit chronic disability .
RESOURCES
• Lung Foundation Australia bit . ly / 3ULv5si
References Available on request from howtotreat @ adg . com . au