Case-Based Review

Management Challenges in Sarcoidosis

Journal of Clinical Outcomes Management. 2016 June;23(6)

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It is important that all patients with confirmed diagnosis of sarcoidosis have an electrocardiogram to exclude cardiac conduction defect(s) irrespective of symptoms. As the patient had a benign Holter profile, we did not persue a cardiac positron-emission tomography (PET). Cardiac PET-CT may have a role in the evaluation for cardiac sarcoidosis with good sensitivity [7,8]. However, it is nonspecific, and positive uptake can be seen in other inflammatory diseases affecting the myocardium, makingit a less reliable test to confirm cardiac sarcoidosis. Cardiac PET has a better prognostic rather than diagnostic value as evidenced by a study by Blankstein et al [9] demonstrating approximately a fourfold increased risk of death or ventricular tachycardia (VT) in the following year if there was evidence of focal perfusion defects and FDG uptake on cardiac PET scan. In our opinion, the utility of PET imaging will increase and further multi-center studies would highlight the potential benefits of this diagnostic modality in the evaluation of cardiac sarcoidosis. The frequency of organ involvement in sarcoidosis is shown in the Table .

Case Continued

The patient was started on corticosteroid eye drops and steroid ointment for his ophthalmologic and cutaneous sarcoidosis, respectively, and symptoms gradually improved over the next few months. As the patient was symptomatic with cough and breathlessness and there was evidence of reduction in FVC (along with reduced DLco), a trial of oral corticosteroids was considered to treat the pulmonary sarcoidosis.

What is first-line pharmacological treatment for sarcoidosis and when is it indicated?

Treatment of sarcoidosis is dependent upon the severity of disease and organ involvement at the time of the diagnosis. Glucocorticoids have traditionally been considered first-line pharmacological agents in selective cases, as a significant proportion of patients do not require drug treatment due to the propensity for spontaneous remission. Furthermore, sarcoidosis remains stable without anti-inflammatory/immunosuppressive therapies in a majority of patients. A number of clinical trials have evaluated the value of corticosteroids in the management of sarcoidosis, with variable outcomes [10–16]. The disease tends to be severe in patients of African descent compared with patients from other racial backgrounds. The European cohort of sarcoidosis patients generally have milder disease with less propensity for vital organ involvement such as cardiac or central nervous system (CNS) disease.

The decision to initiate corticosteroids for sarcoidosis is not a straightforward one as there is variability in symptom presentation, disease severity, and response to corticosteroids. We initiate first-line therapy with oral prednisolone in the following circumstances:

Evidence of pulmonary impairment (forced vital capacity FVC < 80% predicted) with or without reduction in gas transfer (DLco) along with respiratory symptoms of cough, chest pain, and/or breathlessness (as seen in the case patient)
Vital organ involvement such as cardiac, ophthalmic (such as panuveitis) or CNS sarcoidosis once confirmed by respective investigations
Selective cases of sarcoid-associated pulmonary hypertension (SAPH) along with close liaison with pulmonary hypertension specialists

We recommend an initial starting dose of 20 to 40 mg of prednisolone for a period of 1 to 3 months, followed by maintenance dose of 10 mg or less for a further 6 to 9 months, aiming for a total duration of treatment of 12 months. However, the duration may vary depending on the response and any associated adverse effects with corticosteroids. It is usual practice to supplement with calcium and vitamin D when beginning patients on oral corticosteroids due to the potential risk of osteoporosis. However, this may result in significant hypercalcemia, which itself may be an endocrine manifestation of sarcoidosis. Hence, we recommend monitoring serum calcium during treatment and supplement vitamin D in patients who are vitamin D–deficient [17]. Furthermore, serum vitamin 1,25(OH) ₂ vitamin D3 has been demonstrated to the best available test to evaluate vitamin D status in sarcoidosis [18].