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Sarcoidosis
Sarcoidosis is a multisystem inflammatory disease of unknown etiology that predominantly affects the lungs and intrathoracic lymph nodes
Prevalence
1–2 / 10,000
33,100–66,200
US Estimated
51,350–102,700
Europe Estimated
Age of Onset
Adults
ICD-10
D86.9
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
It is most often defined by the presence of noncaseating granulomatous inflammation that occurs in the absence of infection, exposures, malignancy, or alternative immune-related disease
FACT
Lung and thoracic lymph nodes are most often involved, but any organ can be affected
FACT
Patients with multi-system involvement have a worse prognosis
FACT
The basis of treatment of sarcoidosis is regulation of the heightened immune response and suppression of granulomatous inflammation in order to prevent dangerous interference with organ function
FACT
5
Use of corticosteroids and other immunomodulatory agents is often modeled on how they are used in other autoimmune and inflammatory diseases in which suppression of the immune system is desired
Interest over time
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Common signs & symptoms
Persistent dry cough
Eye or skin manifestations
Peripheral lymph nodes
Fatigue
Weight loss
Fever
Night sweats
Löfgren syndrome.
Current treatments
Treatment depends on disease severity and organ involvement.
Observation (mild, asymptomatic cases)
Corticosteroids (first-line therapy)
Immunosuppressive agents:
- Methotrexate
- Azathioprine
Biologic therapies (e.g., TNF inhibitors in refractory cases)
Clinical trials
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| Efficacy and Safety Study of OATD-01 in Patients With Active Pulmonary Sarcoidosis | This is a Phase 2, randomized, double-blind, placebo-controlled, adaptive, multicenter study to evaluate the efficacy, safety, tolerability, Pharmacodynamics (PD), and Pharmacokinetics (PK) of OATD-01 in the treatment of subjects with active pulmonary sarcoidosis. | Phase 2 | Recruiting | Drug: OATD-01 Drug: Placebo | More Info |
| Cardiac Sarcoidosis Randomized Trial | Prospective randomized controlled trial comparing low dose Prednisone(or Prednisolone)/Methotrexate combination to standard dose Prednisone(or Prednisolone) in patients diagnosed with acute active clinically manifest cardiac sarcoidosis and not yet treated. The Investigators hypothesize that low do... | Phase 3 | Recruiting | Drug: Prednisone or Prednisolone Drug: Methotrexate | More Info |
| Evaluation of TNF-alpha Antagonists (Infliximab) Withdrawal in Sarcoidosis | In severe refractory sarcoidosis not responding to conventional immunosuppressive treatment, the third-line tumor necrosis factor (TNF)-alpha inhibitor infliximab is an alternative. Treatment duration is not known, although it has been suggested that relapse rates after withdrawal could be high. We... | Phase 3 | Active | Drug: STOP arm | More Info |
| A Phase 2 Study of the Safety and Efficacy of Brepocitinib in Adults With Cutaneous Sarcoidosis (BEACON) | This study will evaluate the clinical safety and efficacy of oral brepocitinib in participants with cutaneous sarcoidosis. | Phase 2 | Recruiting | Drug: Oral Brepocitinib Drug: Oral Placebo | More Info |
| RCT of Nintedanib in Fibrotic Sarcoidosis | Sarcoidosis is generally managed with outdoor immune modulatory drugs, most commonly oral steroids and at times drugs like methotrexate or azathioprine as a steroid sparing agent. Around 15-20% of sarcoidosis patient develop fibrosis of the lung parenchyma. The effect of antifibrotics in such patie... | Phase 4 | Recruiting | Drug: Nintedanib Drug: Standard of care | More Info |
| Prednisolone for 12 Versus 6 Months to Treat Pulmonary Sarcoidosis | In this study, the efficacy and safety of treating pulmonary sarcoidosis with 12 months vs. 6 months of prednisolone will be compared. The hypothesis is that longer treatment DURAtion would be more effective in preventing treatment failure or early relapse in SARCoidosis (DURASARC trial). The premis... | Phase 4 | Not yet recruiting | Drug: Prednisolone | More Info |
| Effectiveness of Methotrexate Versus Prednisone as First-line Therapy for Pulmonary Sarcoidosis | This is a prospective, randomized, non-blinded, multi-center, non-inferiority trial designed to compare effectiveness and side-effects of methotrexate versus prednisone as first-line therapy for pulmonary sarcoidosis. | Phase 4 | Active | Drug: Methotrexate Drug: Prednisone | More Info |
| Diagnostic Criteria in Cardiac Sarcoidosis | The purpose of the study is to evaluate the accuracy of the current diagnostic criteria of cardiac sarcoidosis. | Recruiting | Diagnostic_Test: ECG, Echo, laboratory tests, CMR, PET-CT, Biopsy | More Info | |
| Evaluation of the Prognostic Value of PET/MRI in Cardiac Sarcoidosis | Cardiac damage is the second leading cause of death in patients with sarcoidosis, after lung damage. Today's challenge is to diagnose the disease as effectively as possible, and to develop tools for better risk stratification, especially for sudden death, in order to better target therapies and impl... | Recruiting | Diagnostic_Test: PET/MRI | More Info | |
| Comparing Menghini-type Needle and Franseen-type Needle in EBUS-TBNA for Sarcoidosis | This randomized pilot clinical trial aims to examine whether sample collection with Franseen-type needles are effective for the diagnosis of sarcoidosis, as defined by improved sample quality for pathological diagnosis compared to the conventional Menghini-type needle. | Recruiting | Device: endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) | More Info |
References:
Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153–2165. doi:10.1056/NEJMra071714Valeyre D, Prasse A, Nunes H, et al. Sarcoidosis. Lancet. 2014;383(9923):1155–1167. doi:10.1016/S0140-6736(13)60680-7Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med. 2011;183(5):573–581. doi:10.1164/rccm.201006-0865CIStatement on Sarcoidosis. American Thoracic Society (ATS). Am J Respir Crit Care Med. 1999;160(2):736–755.