Sarcoidosis: What Are the Symptoms, Causes, and Treatment Options?
Sarcoidosis is an inflammatory disease that causes small clusters of immune cells called granulomas to form in various organs, most commonly the lungs and lymph nodes. While the exact cause remains unknown, understanding the symptoms, diagnostic process, and treatment options can help you manage this condition effectively. This comprehensive guide covers everything you need to know about sarcoidosis.
Key Takeaways
- Sarcoidosis is a non-contagious inflammatory disease that primarily affects the lungs and lymph nodes but can impact any organ in the body.
- Most cases (50-70%) resolve spontaneously within 2-5 years without requiring treatment.
- Common symptoms include persistent dry cough, shortness of breath, fatigue, and skin rashes, though some people have no symptoms at all.
- Diagnosis typically requires chest imaging, blood tests, and tissue biopsy to confirm the presence of characteristic granulomas.
- Treatment, when needed, usually begins with corticosteroids, with other immunosuppressive medications available for persistent cases.
- Regular monitoring is essential even for mild cases, as the disease can affect new organs over time.
What Is Sarcoidosis and What Causes It?
Quick Answer: Sarcoidosis is an inflammatory disease characterized by the formation of tiny clumps of inflammatory cells (granulomas) in one or more organs. The exact cause is unknown, but it appears to result from an abnormal immune response to an unidentified trigger in genetically susceptible individuals.
Sarcoidosis (pronounced sar-koy-DOH-sis) is a systemic inflammatory disease that can affect virtually any organ in the body. The hallmark of the disease is the formation of granulomas—small clusters of immune cells that group together in response to inflammation. These granulomas can accumulate in organs and interfere with their normal function.
Understanding Granulomas
Granulomas form when the immune system attempts to wall off substances it perceives as foreign or harmful. In sarcoidosis, these granulomas form without any identifiable foreign substance present. The granulomas in sarcoidosis are described as "non-caseating," meaning they don't have the cheesy (caseous) center seen in tuberculosis granulomas.
Which Organs Are Affected?
While sarcoidosis can affect any organ, certain areas are more commonly involved:
- Lungs and lymph nodes: Affected in over 90% of cases
- Skin: Affected in 25-35% of cases
- Eyes: Affected in 20-50% of cases
- Liver: Affected in 50-80% of cases (often without symptoms)
- Heart: Affected in 5-25% of cases (can be serious)
- Nervous system: Affected in 5-15% of cases
What Causes Sarcoidosis?
The exact cause of sarcoidosis remains unknown despite decades of research. Current scientific understanding suggests that the disease develops when:
- A genetically susceptible person is exposed to an unknown environmental trigger
- The immune system mounts an exaggerated response to this trigger
- This abnormal immune response leads to granuloma formation
Scientists have investigated various potential triggers including bacteria (especially Propionibacterium acnes and mycobacteria), viruses, and environmental factors like mold, pesticides, and certain occupational exposures. However, no single cause has been definitively identified, and it's likely that multiple different triggers can initiate the disease in susceptible individuals.
Risk Factors
Certain factors increase the likelihood of developing sarcoidosis:
- Age: Most common between ages 25-45, with a second smaller peak after age 50
- Ancestry: Higher rates in Northern Europeans, African Americans, and Scandinavians
- Sex: Slightly more common in women, especially after age 50
- Family history: Having a close relative with sarcoidosis increases risk by 4-5 times
- Occupation: Some studies suggest increased risk in healthcare workers, firefighters, and those exposed to organic dusts
What Are the Symptoms of Sarcoidosis?
Quick Answer: Sarcoidosis symptoms vary widely depending on which organs are affected. Common symptoms include persistent dry cough, shortness of breath, fatigue, skin rashes, and swollen lymph nodes. Many people have no symptoms at all and discover the condition incidentally during routine chest X-rays.
Sarcoidosis is sometimes called "the great imitator" because its symptoms can mimic many other conditions. The presentation varies significantly from person to person, ranging from no symptoms at all to severe multi-organ involvement.
General Symptoms
Many people with sarcoidosis experience nonspecific symptoms that can occur regardless of which organs are affected:
- Fatigue: The most common symptom, affecting up to 70% of patients, often described as overwhelming tiredness that doesn't improve with rest
- Fever: Usually low-grade
- Night sweats: Sweating during sleep
- Weight loss: Unintentional weight loss
- Malaise: General feeling of being unwell
Organ-Specific Symptoms
🫁 Lung Symptoms
- Persistent dry cough
- Shortness of breath
- Wheezing
- Chest discomfort or pain
- Reduced exercise tolerance
👁️ Eye Symptoms
- Blurred vision
- Eye pain or redness
- Sensitivity to light
- Floaters
- Dry eyes
🔴 Skin Symptoms
- Erythema nodosum (painful red bumps on shins)
- Lupus pernio (purple skin lesions on nose, cheeks, ears)
- Skin nodules or plaques
- Rashes
- Scar tissue changes
💓 Heart Symptoms
- Palpitations or irregular heartbeat
- Shortness of breath
- Chest pain
- Fainting or dizziness
- Swelling in legs
🧠 Neurological Symptoms
- Headaches
- Facial weakness or numbness
- Vision changes
- Hearing loss
- Seizures (rare)
🦴 Joint Symptoms
- Joint pain (arthralgia)
- Joint swelling
- Morning stiffness
- Reduced mobility
- Ankle swelling (common)
Acute vs. Chronic Presentation
Löfgren syndrome is an acute form of sarcoidosis characterized by the classic triad of:
- Erythema nodosum (painful red nodules on the shins)
- Bilateral hilar lymphadenopathy (enlarged lymph nodes in the chest)
- Arthritis (especially in the ankles)
This presentation is most common in young women and Northern Europeans. Despite its dramatic onset, Löfgren syndrome has an excellent prognosis, with over 90% of patients experiencing complete resolution within 2 years.
Chronic sarcoidosis develops more insidiously and may involve multiple organs over time. It tends to be more common in African Americans and may require long-term treatment.
How Is Sarcoidosis Diagnosed?
Quick Answer: Diagnosing sarcoidosis requires demonstrating the presence of non-caseating granulomas in affected tissue while excluding other causes. This typically involves chest imaging, blood tests, pulmonary function tests, and tissue biopsy. There is no single definitive test for sarcoidosis.
Sarcoidosis can be challenging to diagnose because its symptoms overlap with many other conditions, and there's no single blood test or imaging study that can confirm the diagnosis. A thorough evaluation typically involves multiple steps.
Initial Evaluation
Medical History and Physical Examination
Your doctor will ask about your symptoms, their duration, and any relevant medical history. The physical examination may reveal:
- Skin lesions or rashes
- Enlarged lymph nodes
- Abnormal lung sounds
- Enlarged liver or spleen
- Eye abnormalities
Imaging Studies
Chest X-ray
Often the first test performed, chest X-rays can reveal characteristic findings in sarcoidosis. The disease is staged based on chest X-ray appearance:
| Stage | Findings | Spontaneous Remission Rate |
|---|---|---|
| Stage 0 | Normal chest X-ray | N/A (extrapulmonary disease) |
| Stage I | Bilateral hilar lymphadenopathy (BHL) only | 55-90% |
| Stage II | BHL with pulmonary infiltrates | 40-70% |
| Stage III | Pulmonary infiltrates without BHL | 10-20% |
| Stage IV | Pulmonary fibrosis with volume loss | 0% |
CT Scan
High-resolution CT (HRCT) of the chest provides more detailed images and can detect:
- Small nodules along lymphatic pathways
- Ground-glass opacities
- Early fibrosis
- Enlarged lymph nodes not visible on X-ray
Blood Tests
While no blood test is diagnostic of sarcoidosis, several tests help assess disease activity and rule out other conditions:
- Complete blood count (CBC): May show anemia or low white blood cell count
- Comprehensive metabolic panel: Checks kidney and liver function
- Calcium levels: Elevated in 10-20% of patients
- Angiotensin-converting enzyme (ACE): Elevated in 60-75% of patients with active disease
- Vitamin D levels: 1,25-dihydroxyvitamin D may be elevated
- Inflammatory markers: ESR and CRP may be elevated
While ACE levels are commonly measured in sarcoidosis, they are neither sensitive nor specific enough for diagnosis. ACE can be elevated in other conditions and normal in many sarcoidosis patients. ACE is most useful for monitoring disease activity in patients with known sarcoidosis who have elevated levels at diagnosis.
Pulmonary Function Tests
These breathing tests measure how well your lungs work and typically show:
- Reduced lung capacity (restrictive pattern)
- Decreased diffusing capacity (DLCO)
- Sometimes airway obstruction
Tissue Biopsy
Definitive diagnosis usually requires tissue biopsy showing non-caseating granulomas. Common biopsy sites include:
- Bronchoscopy with transbronchial biopsy: Most common approach for lung sarcoidosis
- Lymph node biopsy: Via mediastinoscopy or endobronchial ultrasound (EBUS)
- Skin biopsy: When skin lesions are present
- Minor salivary gland biopsy: Sometimes used as it's less invasive
Additional Testing
Depending on symptoms and suspected organ involvement, additional tests may include:
- Eye examination: Slit-lamp examination by an ophthalmologist
- Electrocardiogram (ECG) and echocardiogram: To assess heart function
- Cardiac MRI or PET scan: If cardiac sarcoidosis is suspected
- Lumbar puncture: If neurosarcoidosis is suspected
- 24-hour urine calcium: To assess for hypercalciuria
When Should You See a Doctor About Sarcoidosis?
Quick Answer: See a doctor if you experience persistent unexplained symptoms such as dry cough lasting more than 2-3 weeks, progressive shortness of breath, unexplained fatigue, skin rashes, or eye problems. Seek immediate medical attention for symptoms suggesting cardiac involvement like fainting, severe palpitations, or chest pain.
Many people with sarcoidosis have mild symptoms or no symptoms at all. However, certain signs and symptoms warrant prompt medical evaluation.
See a Doctor If You Have:
- Persistent dry cough lasting more than 2-3 weeks
- Shortness of breath, especially if worsening
- Unexplained fatigue that doesn't improve with rest
- Unexplained weight loss
- Painful red bumps on your shins (erythema nodosum)
- Skin changes or rashes that don't heal
- Eye redness, pain, or vision changes
- Swollen, painful joints (especially ankles)
- Persistent fever or night sweats
Call emergency services or go to the emergency room if you experience:
- Severe chest pain
- Fainting or near-fainting episodes
- Severe palpitations or irregular heartbeat
- Severe difficulty breathing
- Sudden vision loss
- Confusion or severe headache
- Seizures
These symptoms may indicate cardiac sarcoidosis or neurosarcoidosis, which require urgent evaluation.
If You've Been Diagnosed with Sarcoidosis
Contact your doctor if you notice:
- New or worsening symptoms
- Symptoms in a new organ system
- Side effects from medications
- Signs of infection while on immunosuppressive therapy
- Symptoms of depression or anxiety
How Is Sarcoidosis Treated?
Quick Answer: Treatment for sarcoidosis depends on which organs are affected and how severe the disease is. Many patients need no treatment as the disease resolves on its own. When treatment is needed, corticosteroids are the first-line therapy. Other immunosuppressive medications are used for patients who don't respond to steroids or can't tolerate them.
The decision to treat sarcoidosis is based on several factors, including which organs are affected, the severity of symptoms, whether the disease is progressing, and whether organ function is threatened.
When Is Treatment Needed?
Not everyone with sarcoidosis requires treatment. Treatment is typically recommended for:
- Progressive lung disease with declining pulmonary function
- Cardiac involvement
- Neurological involvement
- Eye disease not responsive to topical treatment
- Symptomatic hypercalcemia (elevated calcium)
- Significant symptoms affecting quality of life
- Disfiguring skin disease
Treatment Options
First-Line: Corticosteroids
Prednisone or prednisolone remains the cornerstone of sarcoidosis treatment. Typical starting doses are 20-40 mg daily, gradually tapered over 6-12 months. Side effects include weight gain, diabetes, osteoporosis, and mood changes.
Second-Line: Steroid-Sparing Agents
For patients who need long-term treatment or can't tolerate steroids:
- Methotrexate: Most commonly used steroid-sparing agent
- Azathioprine: Alternative to methotrexate
- Leflunomide: Another option, especially for skin disease
- Mycophenolate: Sometimes used for refractory cases
Third-Line: Biologic Agents
For severe or refractory disease:
- Infliximab: TNF-alpha inhibitor, most evidence for efficacy
- Adalimumab: Another TNF-alpha inhibitor
Organ-Specific Treatments
Additional treatments based on organ involvement:
- Eyes: Steroid eye drops, injections
- Heart: Pacemaker or defibrillator for arrhythmias
- Skin: Topical steroids, hydroxychloroquine
- Lungs: Pulmonary rehabilitation, supplemental oxygen
Monitoring During Treatment
Regular monitoring is essential during treatment:
- Pulmonary function tests every 3-6 months
- Chest X-rays or CT scans periodically
- Blood tests to monitor for medication side effects
- Eye exams annually or more frequently
- Bone density scans if on long-term steroids
Hydroxychloroquine (Plaquenil) is particularly useful for skin sarcoidosis, hypercalcemia, and fatigue. It's generally well-tolerated but requires regular eye exams to monitor for rare retinal toxicity. It's often used in combination with other treatments or as a steroid-sparing agent.
What Is the Prognosis for Sarcoidosis?
Quick Answer: The prognosis for sarcoidosis is generally good. Most people (50-70%) experience spontaneous remission within 2-5 years. About 10-30% develop chronic disease requiring ongoing treatment, and a small percentage develop serious complications. Early-stage disease, Löfgren syndrome, and younger age at diagnosis are associated with better outcomes.
The outcome of sarcoidosis varies considerably between individuals. Several factors help predict how the disease will progress.
Factors Associated with Good Prognosis
- Acute onset (Löfgren syndrome)
- Stage I disease (hilar lymphadenopathy only)
- Younger age at diagnosis (under 40)
- Caucasian or Asian ancestry
- Erythema nodosum at presentation
- Limited organ involvement
Factors Associated with Poorer Prognosis
- Chronic, insidious onset
- Stage III or IV disease
- Older age at diagnosis (over 40)
- African American ancestry
- Cardiac or neurological involvement
- Lupus pernio (skin lesions on nose/face)
- Progressive pulmonary fibrosis
- Hypercalcemia
Long-Term Outcomes
| Outcome | Percentage | Description |
|---|---|---|
| Complete Remission | 50-70% | Disease resolves within 2-5 years, often without treatment |
| Chronic Stable Disease | 20-30% | Ongoing disease controlled with treatment, stable function |
| Progressive Disease | 10-20% | Worsening despite treatment, may develop fibrosis |
| Mortality | 1-5% | Usually from cardiac, pulmonary, or neurological complications |
Relapse
Even after initial remission, sarcoidosis can relapse. Relapses are most common:
- Within the first 2-3 years after stopping treatment
- In patients who required treatment initially
- In patients with chronic disease
Most relapses respond to reinitiation of treatment, though they may indicate a need for longer-term therapy.
How Can You Live Well with Sarcoidosis?
Quick Answer: Living well with sarcoidosis involves maintaining regular medical follow-up, adopting a healthy lifestyle, managing fatigue through energy conservation techniques, staying physically active within your limits, and seeking support for the emotional aspects of living with a chronic condition.
While sarcoidosis can be challenging, many people with the condition lead full, active lives. Here are strategies that can help.
Medical Management
- Keep regular appointments with your healthcare team
- Take medications as prescribed and discuss any side effects
- Get recommended vaccinations, especially if on immunosuppressive therapy
- Have regular eye exams even if you have no eye symptoms
- Report new symptoms promptly to your doctor
Managing Fatigue
Fatigue is one of the most challenging aspects of sarcoidosis. Strategies that may help include:
- Pace yourself: Spread activities throughout the day
- Prioritize tasks: Focus on what's most important
- Rest strategically: Short rests can help, but too much rest can worsen fatigue
- Exercise regularly: Moderate exercise can actually improve energy levels
- Sleep hygiene: Maintain regular sleep schedules
- Address depression: Fatigue and depression often coexist
Lifestyle Recommendations
- Don't smoke: Smoking worsens lung disease
- Avoid excessive sun exposure: Sarcoidosis can cause elevated vitamin D and calcium
- Eat a balanced diet: Limit calcium and vitamin D supplements unless advised otherwise
- Stay active: Regular exercise improves lung function and mood
- Maintain healthy weight: Especially important if on corticosteroids
- Limit alcohol: Especially if on methotrexate or with liver involvement
Emotional Well-being
Living with a chronic condition can affect mental health. Consider:
- Support groups: Connecting with others who have sarcoidosis
- Counseling: Professional help for anxiety or depression
- Stress management: Meditation, yoga, or other relaxation techniques
- Education: Learning about your condition can reduce anxiety
- Communication: Share your needs with family and friends
Work and Disability
Many people with sarcoidosis continue to work. Consider:
- Discussing workplace accommodations with your employer
- Flexible scheduling or work-from-home options for fatigue
- Understanding your rights under disability laws
- Consulting with a social worker if you need disability assistance
Frequently Asked Questions About Sarcoidosis
Medical References
- Crouser ED, Maier LA, Wilson KC, et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;201(8):e26-e51. doi:10.1164/rccm.202002-0251ST
- Baughman RP, Valeyre D, Korber M, et al. ERS Clinical Practice Guidelines on Treatment of Sarcoidosis. Eur Respir J. 2021;58(6):2004079. doi:10.1183/13993003.04079-2020
- Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Müller-Quernheim J. Sarcoidosis. Lancet. 2014;383(9923):1155-1167. doi:10.1016/S0140-6736(13)60680-7
- Judson MA. The Clinical Features of Sarcoidosis: A Comprehensive Review. Semin Respir Crit Care Med. 2020;41(5):601-617. doi:10.1055/s-0040-1713001
- Grunewald J, Grutters JC, Arkema EV, Saketkoo LA, Moller DR, Müller-Quernheim J. Sarcoidosis. Nat Rev Dis Primers. 2019;5(1):45. doi:10.1038/s41572-019-0096-x
- Kirkland CK, Kirkland JL, Mostow EN, Baughhman RP. Treatment of Sarcoidosis: An Evidence-Based Review. Expert Rev Respir Med. 2023;17(2):121-136. doi:10.1080/17476348.2023.2175676
- Foundation for Sarcoidosis Research. Sarcoidosis Quick Facts. Updated 2024. https://www.stopsarcoidosis.org
About Our Medical Editorial Team
Medically Reviewed
All content is reviewed by licensed physicians specializing in pulmonology and internal medicine with expertise in sarcoidosis and granulomatous diseases.
Evidence-Based
Content follows ATS/ERS/WASOG guidelines and is based on systematic reviews, meta-analyses, and peer-reviewed research.
Independent
No pharmaceutical company sponsorship or commercial funding. We maintain complete editorial independence.