Objective: To discuss the management of sarcoidosis.
Methods: Review of the literature.
Results: Sarcoidosis is a challenging multisystem disorder of uncertain etiology characterized by granulomatous inflammation in the affected organs. Treatment is dependent on the severity of disease and organ involvement at the time of diagnosis. Glucocorticoids have traditionally been considered first-line pharmacologic treatment; however, a significant proportion of patients do not require drug treatment due to the propensity toward spontaneous disease remission. Treated patients who fail to respond to corticosteroids or develop significant adverse effects can be offered a second-line agent, eg, methotrexate. Anti-TNF therapy may be considered as a treatment option in carefully selected patients with refractory disease after discussion of potential adverse effects followed by close monitoring at a specialist center.
Conclusion: Further research into therapeutic options is likely to unveil novel agents with different mechanisms of action and better safety profiles than those seen with currently available immunosuppressive regimens.
Sarcoidosis is a multisystem disorder of uncertain etiology characterized by granulomatous inflammation in the affected organs. The diagnosis of sarcoidosis is best supported by histological evidence of noncaseating granuloma formation. It is a disease with a generally good prognosis; less than 5% patients die from the disease, with cause of death usually secondary to respiratory failure or cardiac or neurologic involvement. This review aims to discuss the management of sarcoidosis with a special emphasis on the management challenges resulting from the myriad clinical manifestations and potential complications seen in this chronic multisystem disease.
Case Study
Initial Presentation
A 40-year-old African-American man presents to his primary care physician with symptoms of fatigue, dry cough, exertional breathlessness, dry and painful eyes, generalized arthralgia, and multiple skin lesions for 3 months. He has a history of essential hypertension and is a former smoker with 10 pack-year history. He is not on any regular medications. Examination reveals bilateral cervical lymphadenopathy and multiple skin lesions on trunk. The rest of the systemic examination (including respiratory and cardiovascular system) is normal.
Workup
The primary care physician referred the patient to a pulmonologist for further investigations. A chest radiograph ( Figure 1 ) demonstrated bilateral hilar enlargement. There was no abnormality on wrist and hand radiographs. Pulmonary function tests demonstrated slight reduction in forced expiratory volume in 1 second (FEV 1) at 78% predicted and reduced forced vital capacity (FVC) at 74% predicted with normal FEV 1/FVC ratio, which was consistent with a restrictive ventilatory defect. Diffusing capacity of the lung for carbon monoxide (DLco) was reduced at 60% predicted. A high-resolution CT scan of the thorax ( Figure 2 ) confirmed bilateral symmetrical hilar lymphadenopathy along with
peri-hilar fibrotic change. In addition, there were tiny nodular opacities in both lungs. These radiological features pointed towards a possible diagnosis of sarcoidosis.
The patient’s initial bloodwork showed a mild degree of lymphopenia (1.1 × 10 9/L, normal range 1.5–4.5). Other bloodwork results, bone profile and immunology screen (including ANA, rheumatoid factor, immunoglobulins, and extractable nuclear antigen antibodies) were negative. Angiotensin-converting enzyme (ACE) was elevated at 149 U/L (normal range 5–58), while serum calcium and vitamin D levels (including vitamin D3) were normal. The findings on CT scan along with the biochemical profile suggest a plausible diagnosis of sarcoidosis.
