What is a summary of sarcoidosis in bullet points?

From the Guidelines

Sarcoidosis is a multisystem inflammatory disorder that should be treated based on the risk of death, permanent disability, and impact on quality of life (QoL) 1. The disease is characterized by the formation of granulomas, which are collections of inflammatory cells, primarily affecting the lungs and lymph nodes but capable of involving any organ system.

Key Characteristics of Sarcoidosis

• The disease typically affects adults between 20-40 years of age, with a higher prevalence in African Americans and Northern Europeans.
• Sarcoidosis often presents with non-specific symptoms including fatigue, weight loss, fever, and respiratory symptoms such as cough, shortness of breath, and chest pain.
• Diagnosis requires clinical suspicion combined with histological evidence of non-caseating granulomas and exclusion of other granulomatous diseases.
• Pulmonary involvement occurs in over 90% of cases, manifesting as hilar lymphadenopathy and interstitial lung disease on chest imaging.
• Extrapulmonary manifestations include skin lesions (erythema nodosum, lupus pernio), eye involvement (uveitis), cardiac abnormalities, neurological complications, and hypercalcemia.

Treatment Approach

• Treatment depends on disease severity, with mild cases often requiring only observation as spontaneous remission occurs in many patients.
• For symptomatic or progressive disease, corticosteroids like prednisone (starting at 20-40mg daily with gradual tapering) remain first-line therapy 1.
• Steroid-sparing agents such as methotrexate, azathioprine, hydroxychloroquine, or TNF inhibitors may be used for maintenance therapy or in steroid-resistant cases.

Prognosis and Outcome

• The prognosis is generally favorable, with most patients experiencing disease resolution within 2-5 years, though 10-30% develop chronic disease with potential organ dysfunction.
• Less than 10% of patients die, mostly from advanced lung disease 1.
• The outcome of the disease is variable, and treatment decisions require an assessment of organ involvement, risk for significant morbidity, and impact on QoL of the disease and treatment 1.

From the FDA Drug Label

Symptomatic sarcoidosis

• Sarcoidosis is a condition for which prednisone is indicated
• It is listed under Respiratory Diseases
• The drug is used for symptomatic cases, implying it is used to manage symptoms of the disease 2

From the Research

Sarcoidosis Overview

• Sarcoidosis is a multisystemic disorder characterized by the formation of immune granulomas in involved organs 3
• The disease has an unknown cause, but the prevailing hypothesis is that unidentified antigens trigger an exaggerated immune reaction in genetically susceptible hosts 3
• Sarcoidosis can affect various organs, with the lung and lymphatic system being predominantly affected, but other organs such as the heart, nervous system, eyes, kidneys, and larynx can also be involved 3

Clinical Manifestations

• Common symptoms include persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever, and night sweats 3
• Abnormal metabolism of vitamin D3 and hypercalcemia can also occur 3
• Chest radiography is abnormal in about 90% of cases, showing lymphadenopathy and/or pulmonary infiltrates, which can define sarcoidosis stages from I to IV 3

Diagnosis and Treatment

• Diagnosis relies on compatible clinical and radiographic manifestations, evidence of non-caseating granulomas, and exclusion of other granulomatous diseases 3
• Systemic corticosteroids are the mainstay of treatment, with a minimal duration of 12 months 3, 4
• Alternative treatments, such as immunosuppressive drugs and aminoquinolins, may be useful in case of unsatisfactory response to corticosteroids or poor tolerance 3, 5, 6
• Methotrexate is generally the preferred agent for treatment of chronic sarcoidosis when corticosteroids have inadequate efficacy and/or severe adverse effects 5

Osseous Manifestations

• Bone involvement is less frequent, usually occurring in patients with chronic multisystem disease, and can cause severe disability 7
• Osseous involvement can be asymptomatic, but imaging techniques are important for diagnosis, and biopsy is required for differential diagnosis with respect to malignancy 7
• Treatment of osseous manifestations is part of systemic therapy and may include glucocorticoids and TNF-α antagonists 7