30 HOW TO TREAT : SARCOIDOSIS
30 HOW TO TREAT : SARCOIDOSIS
19 APRIL 2024 ausdoc . com . au
Figure 4 . CT pulmonary angiogram performed in a 74-year-old male with known sarcoidosis for acute progression of dyspnoea . This CT demonstrates advanced and active sarcoidosis and parenchymal changes consistent with the development of fixed fibrosis , as well as ground-glass changes suggestive of active inflammation .
Incidental and asymptomatic mediastinal and hilar lymph node
< 10mm short axis , absence of malignant features
> 10mm short axis
No further evaluation required Known aetiology
No known aetiology
Manage as per condition
Referral to respiratory physician
Observe symptom and is experienced by 50-70 % of those with the condition . 25 The pathophysiology is poorly understood but likely reflects the persistence of circulating inflammatory cytokines . Fatigue is a challenging symptom in the general practice setting because of its wide differential diagnosis and the frustration it creates for patients . Evaluation for other causes of fatigue is important given the multitude of differential diagnoses both related and unrelated to other organ involvement from sarcoidosis . A proposed approach to the investigation and management of fatigue in sarcoidosis is based on the consideration of multiple contributors across the domains of metabolic abnormalities , psychosocial conditions , disease-related fatigue and treatment-induced fatigue . 25
Management with non-pharmacological exercise training programs is suggested as initial therapy , with the addition of stimulant or anti-inflammatory therapies as second- and third-line agents . 20 Evaluation for sarcoidosis fatigue is important in the holistic management of the condition as it is strongly associated with quality of life . 26
Figure 9 offers a management algorithm for sarcoidosis-associated fatigue .
PROGNOSIS
THE prognosis for sarcoidosis is good , with spontaneous remission occurring in up to 70 % of cases , while approximately 10-30 % have chronic or progressive disease requiring specific intervention . 7 Sarcoidosis is described to have fatal sequelae in 1-5 % of cases . However , in the Australian context , this data are likely inflated given people of Afro-Caribbean descent typically have more severe disease , and represent a small proportion of the Australian population . Importantly , in Australia , it is poorly understood if Indigenous Australians experience a similar phenomenon of elevated disease severity .
CASE STUDIES
Case study one
SARA , a 29-year-old Caucasian female , presents to ED with chest pain thought to be costochondritis . Examination and pulmonary function tests were normal . As part of the assessment and to rule out pulmonary emboli , a CT pulmonary angiogram is performed , which demonstrates mediastinal and hilar
Box 5 . Glucocorticoid side effects
• Early onset ( days to weeks ): — Mood lability . — Psychosis . — Insomnia . — Glucose intolerance .* — Fluid retention . — Impaired wound healing .
• Late onset ( weeks to months ): — Higher risk of typical and opportunistic infection — for example , Pneumocystis jiroveci pneumonia . — Osteoporosis .* — Obesity . — Hypertension . — Skin thinning . — Bruising . — Ocular complications , such as cataracts and glaucoma .* — Steroid myopathy . — Adrenal insufficiency .* — Premature atherosclerosis . — Gastritis / gastric ulceration .
* May persist despite cessation of corticosteroids
Box 6 . Cardiac sarcoidosis prognostic variables
• Age older than 50 .
• Left ventricular ejection fraction less than 40 %.
• New York Heart Association class III or IV .
• Increased left ventricular enddiastolic diameter .
• Late gadolinium enhancement on cardiac MRI .
• Ventricular tachycardia .
• Cardiac inflammation identified by FDG – PET scan .
• Echocardiographic evidence of abnormal global longitudinal strain .
• Interventricular septal thinning .
• Elevated troponin or brain natriuretic peptide .
lymphadenopathy without parenchymal changes or pulmonary emboli ( see figure 10 ). She is referred for EBUS , which demonstrates a non-necrotising granuloma , and a diagnosis of sarcoidosis is made .
Sara is advised regarding symptoms to monitor for in the event of disease progression . To exclude extrapulmonary sarcoidosis , she undergoes an ECG and a 24-hour urine collection for hypercalciuria and is referred for an ophthalmic assessment . No sarcoidosis-specific therapy is required , but a plan
Figure 5 . Investigation and follow-up of incidental mediastinal / hilar lymphadenopathy .
Figure 6 . EBUS image demonstrating a mediastinal lymph node with septation ( arrow ), which is often suggestive of sarcoidosis .
Table 3 . Considerations for second-line immunosuppressing agents Steroid-sparing agent Methotrexate
Azathioprine
Mycophenolate mofetil
Infliximab / adalimumab
Rituximab
Tofacitinib
Considerations
for clinical follow-up in 12 months is enacted .
This case reflects most cases seen in Australia , where an incidental finding of mediastinal / hilar lymphadenopathy results in a diagnosis of sarcoidosis , which does not require any further intervention . In this case , knowledge of the diagnosis alleviates anxiety , excludes the differential diagnosis of lymphoma and , in the event of disease progression or multi-organ involvement later in life , means prompt therapy can be provided .
Prescribe once weekly or daily folate for all patients Signs of toxicity — such as infection , pneumonitis , hepatotoxicity — should prompt immediate cessation and consultation with the specialist prescribing the medication
Usually requires a thiopurine methyltransferase to be performed before use to assess for toxicity risk Signs of toxicity include infection and hepatotoxicity , particularly hepatic sinusoidal obstruction syndrome — this is indicated by rapidly rising transaminase levels
May cause diarrhoea Elevated risk of infections
Increases risk of infection Contraindicated in moderate to severe cardiac failure , recent history of malignancy and demyelinating conditions Screening for latent TB is required before administration Antibodies to drug may develop and require biosimilar switching
Screening for viral hepatitis is recommended Can cause long-term hypogammaglobulinaemia ( low IgG )
Elevated risk of infection , malignancy and gastrointestinal perforation
Case study two
Vanessa , a 35-year-old Caucasian female , presents to her GP with a persistent cough . This has been present for the past three months following a mild SARS-CoV-2 infection . She has known sarcoidosis with ocular manifestation that has been indolent for 10 years without immunosuppressant therapy . Her GP organises chest imaging ( see figures 11 and 12 ) and specialist referral .
Given the previously indolent nature of her condition and
Further imaging
Biopsy
extensive nature of the new changes , a bronchoscopy and transbronchial biopsy are performed , which demonstrate non-necrotising granuloma . Oral glucocorticoid is started , and Vanessa ’ s symptoms promptly resolve .
This is a unique case of a sarcoidosis flare in the context of a SARS-CoV-2 infection ; there is evidence that SARS-CoV-2 can induce de novo sarcoidosis in some cases . 27
Case study three
Andrew , a 39-year-old PAGE 32