What is the common presentation of sarcoidosis?

From the Guidelines

Sarcoidosis commonly presents as a young African American female with bilateral hilar lymphadenopathy on chest X-ray, often accompanied by erythema nodosum and bilateral ankle arthritis, known as Löfgren syndrome, which typically has a good prognosis. This classic presentation is supported by the American Thoracic Society clinical practice guideline 1. Other key presentations include:

• Pulmonary symptoms like dry cough, dyspnea, and chest pain
• Hypercalcemia due to increased vitamin D production by activated macrophages
• Non-caseating granulomas on biopsy
• Ocular manifestations (uveitis)
• Skin lesions (lupus pernio)
• Cardiac involvement (arrhythmias, heart block)
• Neurological symptoms Laboratory findings often include elevated angiotensin-converting enzyme (ACE) levels, though this is not specific 1. Diagnosis typically requires compatible clinical and radiographic findings plus histological evidence of non-caseating granulomas with exclusion of other causes. Treatment depends on organ involvement, with corticosteroids being first-line therapy for symptomatic disease, usually prednisone 20-40mg daily for 6-12 months with gradual tapering, as recommended by the European Respiratory Review 1. The pathophysiology involves an exaggerated immune response to an unknown antigen, leading to granuloma formation in affected tissues. In cases of cardiac involvement, cardiac MRI with late gadolinium enhancement is considered the imaging study of choice, and corticosteroid treatment is commonly initiated at a high dose and tapered off slowly over a period of months if clinical and imaging features remain stable or improve 1.

From the Research

Common Presentation of Sarcoidosis

• The most common clinical symptoms of sarcoidosis include:
  • Dry cough 2, 3, 4
  • Dyspnea 2, 3, 4
  • Chest discomfort 2
  • Eye and skin manifestations 3
  • Weight loss 3
  • Fatigue 3
  • Night sweats 3
  • Erythema nodosum 3
• Sarcoidosis can affect virtually any organ, but intrathoracic involvement is the hallmark of the disease, present in over 90% of patients 5, 4
• The entire respiratory tract can be involved, with the most common areas of involvement being the airways and interstitium 4

Imaging Manifestations

• Lymphadenopathy is a typical imaging manifestation of sarcoidosis 2
• High-resolution computed tomographic (HRCT) scans are superior to chest X-rays in assessing the extent of disease 6
• Typical findings on HRCT include:
  • Micronodules 6
  • Perilymphatic and bronchocentric distribution 6
  • Perihilar opacities 6
  • Varying degrees of fibrosis 6
• Less common findings on CT include:
  • Mass-like or alveolar opacities 6
  • Miliary opacities 6
  • Mosaic attenuation 6
  • Honeycomb cysts 6
  • Cavitation 6

Diagnosis and Treatment

• Diagnosis requires the presence of noncaseating granuloma and compatible presentations after exclusion of other identifiable causes 5
• Glucocorticoids are the cornerstone of treatment of sarcoidosis, even though evidence from randomized controlled studies is lacking 5
• Glucocorticoid-sparing agents and biologic agents are often used as second- and third-line therapy for patients who do not respond to glucocorticoids or experience serious adverse effects 5