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sarcoidosis
ICD-10 Codes
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Description
What is Sarcoidosis?
Sarcoidosis is an inflammatory disease that affects multiple organs in the body, including the lungs, eyes, skin, and heart. It is characterized by the formation of tiny granulomas, which are small groups of inflammatory cells, in various parts of the body [1]. These granulomas can cause a range of symptoms, from mild to severe.
Causes and Symptoms
The exact cause of sarcoidosis is unknown, but it is believed to be related to an abnormal immune response. The disease can affect anyone, but it is more common in people between the ages of 20 and 40 [2]. Symptoms may include:
- Fatigue
- Fever
- Inflammation of the eyes (uveitis)
- Skin lesions or rashes
- Joint pain or swelling
- Respiratory problems, such as coughing or shortness of breath
Organ Affected
Sarcoidosis can affect various organs in the body, including:
- Lungs: The most common site for granulomas to form.
- Eyes: Uveitis is a common symptom.
- Skin: Lesions or rashes may appear.
- Heart: Inflammation of the heart muscle (myocarditis) can occur.
- Liver and lymph glands: Granulomas may also form in these organs.
Treatment and Prognosis
While there is no cure for sarcoidosis, treatment options are available to manage symptoms and prevent complications. These may include medications to reduce inflammation, such as corticosteroids, or immunosuppressive agents [3]. In some cases, surgery may be necessary to remove affected tissue.
Symptoms can go away within a few years without treatment, but in some cases, the disease can progress and cause serious complications [4].
References
[1] Sarcoidosis is an inflammatory disease of unknown origin. The hallmark of sarcoidosis is the formation of nodules, or granulomas, in the lungs and other organs. [2] Sarcoidosis starts with active, ongoing inflammation. Granulomas (lumps) form and grow. Symptoms may go away within a few years without treatment. [3] Sarcoidosis is an inflammatory disease that can produce small clusters of cells called "granulomas" anywhere in or on the body, including internal organs. [4] Symptoms may go away within a few years without treatment.
Additional Characteristics
- fatigue
- fever
- respiratory problems
- inflammatory disease
- affects multiple organs
- characterized by formation of tiny granulomas
- symptoms range from mild to severe
- exact cause unknown
- abnormal immune response believed to be related
- can affect anyone, but more common in people between 20-40 years old
- inflammation of the eyes (uveitis)
- skin lesions or rashes
- joint pain or swelling
Signs and Symptoms
Symptoms of Sarcoidosis
Sarcoidosis, a chronic inflammatory disease, can cause a wide range of symptoms depending on which organs are affected. Here are some common signs and symptoms:
- Respiratory symptoms: Shortness of breath, wheezing, persistent dry cough, and chest pain are common in people with pulmonary sarcoidosis [6].
- Fatigue and weight loss: Many people with sarcoidosis experience fatigue and weight loss due to the disease's inflammatory nature [7].
- Swollen lymph nodes: Enlarged lymph nodes in the center of the chest (hilar lymphadenopathy) are a characteristic sign of sarcoidosis, often accompanied by no symptoms or mild respiratory issues [11].
- Skin manifestations: Sarcoidosis can cause various skin symptoms, including small bumps, raised patches, lumps under the skin, rash, and discolored skin [8][9].
- Eye problems: Some people with sarcoidosis may experience blurred vision, eye pain, or other signs of eye involvement [10].
- Joint pain and fever: Sarcoidosis can cause joint pain, fever, night sweats, and depression in some cases [7].
Organ-specific symptoms
Sarcoidosis can affect various organs, leading to specific symptoms:
- Lung symptoms: Shortness of breath, wheezing, persistent dry cough, and chest pain are common lung-related symptoms.
- Skin symptoms: Small bumps, raised patches, lumps under the skin, rash, and discolored skin are characteristic skin manifestations.
- Eye symptoms: Blurred vision, eye pain, or other signs of eye involvement may occur in people with sarcoidosis.
Stages of Sarcoidosis
Sarcoidosis can progress through different stages:
- Stage 0: No symptoms or mild respiratory issues.
- Stage 1: Enlarged lymph nodes and nodules in the lungs from granulomas, often resolving on its own without treatment [11].
Treatment and Management
Treatment for sarcoidosis depends on the severity of the disease and the symptoms experienced. Medications like corticosteroids (e.g., prednisone) may be prescribed to manage symptoms and inflammation [12].
Additional Symptoms
- Shortness of breath
- Eye pain
- Swollen lymph nodes
- Lumps under the skin
- Persistent dry cough
- Small bumps on skin
- Raised patches on skin
- Rash on skin
- Discolored skin
- weight loss
- chest pain
- blurred vision
- night sweats
- wheezing
- fever
- joint pain
- fatigue
- depression
Diagnostic Tests
Diagnostic Tests for Sarcoidosis
Sarcoidosis diagnosis involves a combination of medical history, physical examination, and various tests to confirm the presence of granulomas in affected organs.
- Imaging Tests: Chest X-ray, CT scans, and MRI with gadolinium are commonly used to diagnose sarcoidosis. These tests help identify granulomas in the lungs, liver, and other organs [3][4].
- Blood and Urine Tests: Blood and urine tests assess overall health and kidney and liver function, which can be affected by sarcoidosis [2].
- Lung Function Tests (PFTs): PFTs measure lung capacity and airflow, helping to diagnose respiratory involvement in sarcoidosis [3].
- Lung Biopsy: A lung biopsy is a definitive test for diagnosing sarcoidosis, as it allows direct examination of granulomas under a microscope [4][8].
Additional Tests
Other tests may be performed to rule out other conditions or assess the severity of sarcoidosis. These include:
- Electrocardiogram (ECG) to evaluate heart function
- High-resolution chest scans to visualize lung involvement
- Pulmonary function tests to measure lung capacity and airflow
References:
[1] Mar 24, 2022 — Sarcoidosis is diagnosed based on symptoms, physical exam, and imaging tests or a biopsy. [2] Jan 30, 2019 — Blood and urine tests assess overall health and kidney and liver function. [3] Common Tests Used to Arrive at Sarcoidosis Diagnosis [4] Sarcoidosis is usually diagnosed with a combination of a physical exam, imaging (such as a chest X-ray) and biopsy of suspected granulomas. [5] The process of diagnosing sarcoidosis involves a medical history, physical exam and multiple tests. [6] According to an international consensus statement, there are three criteria for diagnosing sarcoidosis: (1) a compatible clinical and radiologic presentation. [7] MRI with gadolinium is the preferred imaging modality for diagnosing neurosarcoidosis. [8] Tests to Diagnose Sarcoidosis of the Lung: What to Expect [9] “Currently, diagnosing sarcoidosis isn't a straightforward process, and requires tissue removal and testing with additional screenings to rule out other conditions.
Additional Diagnostic Tests
- Blood tests
- Electrocardiogram (ECG)
- Chest X-ray
- Urine tests
- Pulmonary function tests
- Lung Biopsy
- CT scans
- MRI with gadolinium
- Lung Function Tests (PFTs)
- High-resolution chest scans
Treatment
Treatment Options for Sarcoidosis
Sarcoidosis, a chronic inflammatory disease, can be effectively managed through various drug treatments. The primary goal of treatment is to reduce inflammation and prevent organ damage.
- Corticosteroids: These powerful anti-inflammatory drugs are usually the first line of treatment for sarcoidosis (1, 2). Corticosteroids help to reduce systemic inflammation, thereby slowing or halting disease progression.
- Immunomodulating medications: Chloroquine and hydroxychloroquine, antimalarial drugs with immunomodulating properties, have been used to treat cutaneous lesions and other forms of sarcoidosis (3, 4). These medications can help reduce inflammation and prevent further organ damage.
- Immune system suppressant medication: Methotrexate is an immunosuppressant commonly used in sarcoidosis treatment (5, 6). This medication helps to reduce the immune system's activity, thereby reducing inflammation. Other immune system suppressants like azathioprine, leflunomide, and mycophenolate are also used as steroid-sparing alternatives (7).
- Steroid-sparing medications: Methotrexate is often used in combination with other medications to reduce the need for corticosteroids (8). This approach helps minimize side effects associated with long-term corticosteroid use.
Key Statistics
- According to a study, 23% of patients with sarcoidosis were prescribed vitamins and mineral combinations, while 9.1% received glucocorticoids as part of their treatment regimen (10).
It's essential to note that the effectiveness of these treatments can vary depending on individual patient factors and disease severity. A healthcare provider will work with you to determine the best course of treatment for your specific needs.
References:
(1) Corticosteroids are usually the first line treatment for sarcoidosis. (2) For sarcoidosis patients, corticosteroids are the first line therapy. (3) Chloroquine and hydroxychloroquine are antimalarial drugs with immunomodulating properties, which have been used for cutaneous lesions, ... (4) Chloroquine and hydroxychloroquine (HCQ) are antimalaria drugs used to treat sarcoidosis. Recently, HCQ is used more often due to its lower toxicity profile, ... (5) Corticosteroids, or prednisone, which turn down the immune system's activity to reduce inflammation. (6) Immune system suppressant medication: Because sarcoidosis is an autoimmune disease, medications that suppress the immune system can be effective in treating it. (7) Corticosteroids are also the initial drug of choice to treat most forms of extrapulmonary sarcoidosis. As a general rule, 20–40 mg of daily prednisone ... (8) Methotrexate is an immunosuppressant commonly used in sarcoidosis. Anti-tumor necrosis factor-alpha antibodies (anti-TNF-alpha antibodies): Anti-TNF-alpha ... (9) Methotrexate, azathioprine, leflunomide, and mycophenolate are the most common steroid-sparing alternatives applied to treat sarcoidosis. (10) Result: | male | female | all vitamin and mineral combinations | 23% | 35% | 27% vitamins | 28% | 2.4% | 19% glucocorticoids | 9.1% | 24% | 14%
Recommended Medications
- Corticosteroids
- Immunomodulating medications (Chloroquine, Hydroxychloroquine)
- Immune system suppressant medication (Methotrexate)
- Steroid-sparing medications (Methotrexate, Azathioprine, Leflunomide, Mycophenolate)
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Sarcoidosis
Sarcoidosis, a systemic granulomatous disease, can be challenging to diagnose due to its nonspecific symptoms and diverse clinical presentations. The differential diagnosis for sarcoidosis is broad and includes various conditions that may mimic its presentation.
Granulomatous Disorders
- Mycobacterial infection: Atypical mycobacteria, tuberculosis [4]
- Fungal infection: Aspergillosis, Blastomycosis [5]
Lymphoproliferative Disorders
- Lymphoma: Hodgkin's lymphoma [8]
Infectious Diseases
- Leishmaniasis
- Toxoplasmosis
- Other granulomatous infections [8]
Other Conditions
- Eosinophilic Granuloma [1]
- Cat Scratch Disease (Cat Scratch Fever) [1]
- Diffuse Large B-Cell Lymphoma (DLBCL) [1]
- Berylliosis [1]
The differential diagnosis with sarcoidosis is rarely difficult unless the lung is the only site involved, with condensations or noncavitated nodular lesions [2][9]. A thorough diagnostic workup, including imaging studies and laboratory tests, is essential to rule out these conditions and confirm a diagnosis of sarcoidosis.
References: [1] - Context search result 1 [2] - Context search result 2 [4] - Context search result 4 [5] - Context search result 5 [8] - Context search result 8 [9] - Context search result 9
Additional Differential Diagnoses
- Fungal infection
- Cat Scratch Disease (Cat Scratch Fever)
- Mycobacterial infection
- Eosinophilic Granuloma
- diffuse large B-cell lymphoma
- lymphoma
- berylliosis
- toxoplasmosis
- leishmaniasis
- axillary adenitis
- obsolete leptomeningeal neoplasm
- lymphopenia
- aleukemic leukemia
- lichen nitidus
- submandibular gland cancer
- large B-cell lymphoma
- autoimmune optic neuritis
- obsolete pulmonary blastomycosis
- obsolete pulmonary paracoccidioidomycosis
- systemic mycosis
- idiopathic pulmonary fibrosis
- cryptogenic organizing pneumonia
- granulomatous amebic encephalitis
- penicilliosis
- meningovascular neurosyphilis
- extrapulmonary tuberculosis
- Blau syndrome
- mixed extragonadal germ cell cancer
- MHC class I deficiency
- autoimmune disease of exocrine system
- Cogan syndrome
- erythema elevatum diutinum
- ocular tuberculosis
- adrenocorticotropic hormone deficiency
- glucocorticoid deficiency 1
- Graves ophthalmopathy
- chronic granulomatous disease
- pulmonary fibrosis
- adrenal cortex disease
- salivary gland cancer
- arrhythmogenic right ventricular dysplasia 1
- obsolete arrhythmogenic right ventricular dysplasia 2
- arrhythmogenic right ventricular dysplasia 3
- arrhythmogenic right ventricular dysplasia 4
- arrhythmogenic right ventricular dysplasia 6
- arrhythmogenic right ventricular dysplasia 8
- arrhythmogenic right ventricular dysplasia 9
- arrhythmogenic right ventricular dysplasia 12
- arrhythmogenic right ventricular dysplasia 13
- birdshot chorioretinopathy
- interstitial lung disease
- arthropathy
- lacrimal passage granuloma
- paratesticular lipoma
- leprosy
- parotid disease
- pneumoconiosis
- mixed mineral dust pneumoconiosis
- Caplan's syndrome
- pulmonary talcosis
- orbital granuloma
- obsolete pinna disease
- protein-losing enteropathy
- obsolete nodular lung tuberculosis
- chorioretinal scar
- acute orbital inflammation
- rhinoscleroma
- optic neuritis
- Bell's palsy
- tolosa-hunt syndrome
- Sjogren's syndrome
- allergic bronchopulmonary aspergillosis
- obsolete Histoplasma duboisii pericarditis
- sick sinus syndrome
- lacrimal apparatus disease
- bilateral hyperactive labyrinth
- obsolete secondary malignant neoplasm of intrathoracic lymph node
- histoplasmosis
- larynx cancer
- rheumatoid arthritis
- obsolete histoplasmosis pneumonia
- obsolete Histoplasma capsulatum pneumonia
- mononeuritis
- malignant oculomotor nerve tumor
- pulmonary tuberculosis
- hyperglobulinemic purpura
- granulomatous myositis
- inclusion body myositis
- necrobiosis lipoidica
- mixed connective tissue disease
- obsolete intrathoracic lymph node mast cell malignancy
- pulmonary plasma cell granuloma
- granuloma annulare
- granulomatous gastritis
- akinetic mutism
- granulomatous dermatitis
Additional Information
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- disease_ontology
- oboInOwl#id
- DOID:11335
- core#notation
- DOID:11335
- oboInOwl#hasDbXref
- UMLS_CUI:C0036202
- core#exactMatch
- MESH:D012507
- IAO_0000115
- A hypersensitivity reaction type IV disease characterized by the growth of collections of inflammatory cells (granulomas) in multiple organs.
- oboInOwl#hasExactSynonym
- lymphogranulomatosis
- rdf-schema#label
- sarcoidosis
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#NCIthesaurus
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- relatedICD
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- owl#annotatedSource
- t398366
- 22-rdf-syntax-ns#first
- t403460
- owl#someValuesFrom
- t4020793
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