Pulmonology Book

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Sarcoidosis

Aka: Sarcoidosis, Lupus Pernio, Lofgren's Syndrome, Lofgren Syndrome, Lofgrens syndrome, Heerfordt Syndrome, Uveoparotid Fever
  1. Epidemiology
    1. Affects young adults most commonly ages 20 to 40 years old
      1. Second peak onset 50 to 65 years old
    2. Prevalence in United States
      1. Most common in U.S. Black patients, African-Caribbean, Danes and Swedes
      2. Black patients: 40 per 100,000
      3. White patients: 5 per 100,000
  2. History
    1. First described by Hutchinson in 1877
  3. Pathophysiology
    1. Noncaseating granuloma formation
      1. Activated T Cells and Macrophages secrete cytokines and TNFa
    2. Idiopathic
      1. Underlying genetic predisposition
        1. First-degree relative with Sarcoidosis: 4-10% of patients
      2. Precipitated by trigger
        1. Infection (e.g. Mycobacteria, Borrelia Burgdorferi, Propionibacterium acnes)
        2. Environmental exposure (e.g. Beryllium, Aluminum)
  4. Presentations: Common involvement sites (affects all organ systems)
    1. Lungs (>90%)
      1. Interstitial Lung Disease
      2. Pulmonary Hypertension
    2. Lymphadenopathy
      1. Hilar adnenopathy and mediastinal Lymphadenopathy (>95%)
      2. Cervical Lymphadenopathy (and Supraclavicular Lymphadenopathy)
    3. Liver (50-80%)
      1. Hepatic Granulomas (86%)
      2. Hepatomegaly (20%)
      3. Mild Liver Function Test Abnormality
        1. Increased Alkaline Phosphatase
    4. Spleen (40-80%)
    5. Skin lesions (25%)
      1. Lupus Pernio
      2. Dactylitis
      3. Erythema Nodosum (See Lofgren Syndrome below)
    6. Eyes (20-50%)
      1. Anterior Uveitis (18%, also in Spondyloarthropathy)
      2. Posterior Uveitis (7%, Behcet's Disease)
      3. Lacrimal gland hypertrophy
      4. Conjunctival Nodules
      5. Keratoconjunctivitis (also in Sjogren's Syndrome)
      6. Proptosis (also in Wegener's Granulomatosis)
    7. Heart (5%)
      1. Tachyarrhythmias (including Ventricular Tachycardia)
      2. Cardiomyopathy
      3. Congestive Heart Failure
    8. Kidney (uncommon)
      1. Membranous Glomerulonephritis
      2. Nephrocalcinosis
      3. Nephrolithiasis
      4. Renal Insufficiency
    9. Bone Marrow (4-40%)
      1. Leukopenia (28%)
      2. Eosinophilia (34%)
    10. Gastrointestinal
      1. Pancreas (6%)
      2. Stomach or esophagus
    11. Parotid Gland (5%, also seen in Sjogren's Syndrome)
      1. May be associated with Heerfordt Syndrome
    12. Skeletal muscle (4%, also seen in Polymyositis)
      1. Proximal Muscle Weakness
    13. Upper airway (3%, also in Wegener's Granulomatosis)
      1. Saddle-nose deformity
    14. Nervous System or Neurosarcoidosis (10%)
      1. Atypical central effects (e.g. acute Aphasia)
      2. Peripheral Neuropathy (esp. small fiber)
      3. Cranial Nerve palsy (especially CN 7, Bell's Palsy)
      4. Optic Neuritis
      5. Hypopituitarism (from pituitary and Hypothalamus involvement)
      6. Seizures (due to CNS Lesions)
    15. Hypercalcemia (<10%)
      1. Results from Vitamin D activation with increased intestinal calcium absorption
      2. Nephrocalcinosis
      3. Nephrolithiasis
      4. Renal Failure
  5. Symptoms
    1. Asymptomatic initially in many patients
      1. Often diagnosed by Hilar Adenopathy on Chest XRay
    2. Pulmonary (presenting symptom in 40-45%)
      1. Dry cough
      2. Dyspnea
      3. Chest Pain (non-specific)
      4. Hemoptysis (rare initially)
    3. Constitutional symptoms (presenting symptom in 25%)
      1. Fever (associated with hepatic granulomas)
      2. Weight loss
      3. Fatigue
      4. Malaise
  6. Signs: Arthritis (occurs in 10-15% of cases)
    1. Early joint disease (first 6 months)
      1. Duration: weeks to 3 months
      2. Common joints involved
        1. Onset in ankles
        2. Spreads to knees
      3. Involves other joints
        1. Proximal interphalangeal joint
        2. Metacarpophalangeal joint
        3. Wrist
        4. Elbow
      4. Spares axial skeleton
      5. Associated with Erythema Nodosum
      6. No XRay changes
    2. Late joint disease (onset after 6 months)
      1. Common joints involved
        1. Knees
        2. Ankles
        3. Proximal interphalangeal joints
      2. Associated with chronic cutaneous Sarcoidosis
      3. XRay changes (see below)
  7. Signs: Skin changes (Lupus Pernio)
    1. Initial Characteristics
      1. Papular lesions (most common)
      2. Reddish-brown to purple color (violaceous Plaques)
      3. Scaling may occur
      4. Diameter: 1 to 3 cm
    2. Later Characteristics
      1. Lesions coalesce into Annular Lesions or Plaques
      2. Chronic form may show scarring and disfigurement
    3. Distribution (most commonly involves face)
      1. Periorbital area
      2. Nasolabial folds
      3. Mucous membranes
      4. Ears
      5. Fingers and Toes
    4. Other skin changes
      1. Dactylitis (sausage digits)
        1. Associated with chronic Arthritis
  8. Signs: Lymphadenopathy
    1. Mediastinal lymph node involvement most common
    2. Peripheral Lymphadenopathy (non-tender, <5 cm)
      1. Cervical Lymphadenopathy
      2. Axillary Lymphadenopathy
      3. Inguinal Lymphadenopathy
      4. Epitrochlear Lymphadenopathy
  9. Staging
    1. Based on Chest XRay (see below)
  10. Imaging: Chest XRay (abnormal in 90% of cases)
    1. Stage 0: No abnormality (<10% of cases)
    2. Stage I: Lymphadenopathy alone (43% of cases)
      1. Bilateral hilar Lymphadenopathy
      2. Mediastinal Lymphadenopathy
      3. Right paratracheal Lymphadenopathy
    3. Stage II: Adenopathy and Infiltrates (24% of cases)
      1. Lymphadenopathy as in Type I Chest XRay findings
      2. Parenchymal infiltrates
      3. Symptomatic respiratory disease presentation
    4. Stage III: Infiltrates alone (13% of cases)
      1. Parenchymal infiltrates
    5. Stage IV: Pulmonary Fibrosis
  11. Imaging: Joint and Bone XRay in Arthritis
    1. Acute Arthritis not associated with XRay changes
    2. Chronic Arthritis uncommonly with XRay changes
      1. Middle and distal phalanx bone destruction or cysts
      2. Trabecular changes to bone (honeycombing)
  12. Labs: Diagnosis
    1. Pulmonary Function Testing
      1. Findings consistent with Interstitial Lung Disease
    2. Serum Angiotensin-converting enzyme (Serum ACE)
      1. Not typically recommended (low Test Specificity, variable across patients)
      2. Increased in 50-80% of Sarcoidosis patients
    3. Advanced Imaging
      1. CT Chest
        1. Evaluates differential diagnosis of Interstitial Lung Disease, pulmonary fibrosis, Hilar Adenopathy
      2. F-fluorodeoxyglucose Positron Emission Tomography (F-FDG PET)
        1. Identifies activity distribution as well as biopsy sites, cardiac Sarcoidosis
      3. MRI Brain
        1. CNS Lesions
      4. MRI Heart
        1. Intramyocardial inflammation
        2. Delayed gadolinium enhancement is a risk for ventricular arrhythmia
      5. Cardiac thallium scan
        1. Decreased uptake from sarcoid lesions does not follow Coronary Artery distribution
    4. Gallium scan
      1. Lambda pattern or sign
        1. Bilateral hilar and right paratracheal nodal uptake
      2. Panda pattern or sign
        1. Parotid and lacrimal gland uptake
    5. Biopsy or Cytology (Gold standard)
      1. Protocol
        1. Samples typically obtained via flexible bronchoscopy with biopsy
      2. Finding
        1. Discrete noncaseating epithelioid granuloma
      3. Biopsy sites
        1. Transbronchial lung biopsy (preferred site)
          1. Transbronchial needle aspiration (TBNA)
          2. Ultrasound may guide TBNA
        2. Bronchoalveolar lavage (CD4-CD8 ratio >3.5)
        3. Skin biopsy of lesion
        4. Palpable peripheral lymph node biopsy
        5. Salivary Gland biopsy
  13. Labs: Additional baseline for Sarcoidosis monitoring
    1. Complete Blood Count
    2. Serum Comprehensive Metabolic Panel
      1. Serum Calcium
        1. Hypercalcemia occurs in 2-10% of patients
      2. Renal Function tests
      3. Liver Function Tests
    3. Urinalysis
    4. Tuberculosis Screening
      1. Tuberculosis Antigen-Specific Interferon-Gamma Release Assay (e.g. Quantiferon-TB) - preferred
      2. Tuberculin Skin Test (PPD)
  14. Diagnostics: Other
    1. Electrocardiogram
    2. Echocardiogram
    3. Ophthalmology evaluation
  15. Associated Conditions: Variants
    1. Lofgren Syndrome
      1. Erythema Nodosum (typically presenting complaint, suggests better prognosis)
      2. Bilateral hilar Lymphadenopathy
      3. Fever
      4. Polyarthritis (not typically chronic)
      5. Uveitis
    2. Heerfordt Syndrome (Uveoparotid Fever)
      1. Uveitis
      2. Parotitis
      3. Fever
      4. Facial Nerve Palsy (variably present)
  16. Differential Diagnosis: Pulmonary
    1. See Interstitial Lung Disease
    2. See Hilar Adenopathy
    3. Infections
      1. Tuberculosis
      2. Coccidioidomycosis
      3. Blastomycosis
      4. Aspergillosis
      5. Histoplasmosis
      6. Pneumocystis jiroveci (Pneumocystis carinii)
      7. Brucellosis
    4. Exposures or toxins
      1. See Interstitial Lung Disease
      2. Drug induced hypersensitivity (e.g. Monoclonal AntibodyDMARDs, Methotrexate)
      3. Hypersensitivity pneumonitis
    5. Malignancy
      1. Lymphoma
    6. Vasculitis
      1. Churg-Strauss Syndrome
      2. Wegener Granulomatosis
  17. Differential Diagnosis: Extra-pulmonary
    1. Arthritic Conditions
      1. Rheumatoid Arthritis
      2. Rheumatic Fever
      3. Systemic Lupus Erythematosus
      4. Gonococcal Arthritis
      5. Spondyloarthropathy
    2. Skin Differential Diagnosis
      1. See Annular Lesion
      2. See Erythema Nodosum
      3. Papular lesions similar to Sarcoidosis
        1. Granulomatous Rosacea
        2. Acne Vulgaris
      4. Plaque-type lesions similar to Sarcoidosis
        1. Psoriasis
        2. Lichen Planus
        3. Nummular Eczema
        4. Discoid Lupus Erythematosus
        5. Granuloma Annulare
        6. Cutaneous T-Cell Lymphoma
        7. Kaposi's Sarcoma
        8. Secondary Syphilis
      5. Reference
        1. Katta (2002) Am Fam Physician 65(8):1581-84 [PubMed]
  18. Diagnosis: Criteria
    1. Clinical and imaging findings are consistent with Sarcoidosis AND
    2. Other conditions on differential diagnosis are excluded AND
    3. Noncaseating granulomas on pathology
      1. Biopsy not required in classic Lofgren Syndrome or Heerfordt Syndrome
      2. Early disease (asymptomatic Stage I Sarcoidosis) as management is not affected
  19. Management: Pulmonary Sarcoid
    1. Indications
      1. Dyspnea
      2. Persistent cough
      3. Widespread debilitating disease
    2. First-line: Systemic Corticosteroids (e.g. Prednisone)
      1. Indications
        1. Stage 2 or 3 lung changes
      2. Efficacy
        1. Short term benefit in moderate lung disease
        2. Unclear whether disease-modifying effect
      3. Protocol
        1. Start Prednisone at 20 to 40 mg per day
        2. Evaluate at 1-3 months for response
          1. No response
            1. Taper off over 4-6 weeks
          2. Response
            1. Taper Prednisone to 5-10 mg/day
            2. Continue Prednisone for total of 12 months
        3. Monitoring tools (obtain at 1-3 months, and repeat every 3-6 months while on Prednisone)
          1. Symptom history
            1. Pulmonary symptoms (e.g. cough, Dyspnea)
            2. Prednisone adverse effects
          2. Pulmonary Function Tests
          3. Chest XRay
        4. Osteoporosis Prevention for longstanding Corticosteroid use
          1. See Corticosteroid Associated Osteoporosis
      4. Reference
        1. Paramothayan (2002) JAMA 287:1301-7 [PubMed]
        2. (1999) Am J Respir Crit Care Med 160:736-55 [PubMed]
    3. Other management options
      1. Agents used as alternative or as adjunct to Prednisone
      2. Indications
        1. Corticosteroid refractory disease (10-15 mg/day Prednisone)
        2. Significant Corticosteroid adverse effects
        3. Frequent Sarcoidosis exacerbation
      3. Cytotoxic agents
        1. Methotrexate (Rheumatrex) 10-25 mg weekly
        2. Azathioprine (Imuran)
        3. Leflunamide (Arava)
      4. Immunomodulators
        1. Chloroquine
        2. Hydroxychloroquine (Plaquenil)
      5. Monoclonal Antibodies
        1. Inlfliximab (Remicade)
        2. Adalimumab (Humira)
      6. Thoracic surgery indications
        1. Life-threatening Hemoptysis (lung resection)
        2. End-stage pulmonary Sarcoidosis (lung transplant)
  20. Management: Extrapulmonary Sarcoid
    1. Ophthalmologic Sarcoidosis: Uveitis
      1. First line: Topical Corticosteroids
      2. Refractory cases
        1. Prednisone (preferred)
        2. Methotrexate
    2. Cutaneous Sarcoidosis
      1. Erythema Nodosum lesions: NSAIDs
      2. Sarcoid lesions
        1. Intralesional Corticosteroids (e.g. Kenalog 5/ml)
          1. Inject lesions q2-3 weeks
        2. Other agents
          1. Doxycycline
          2. Minocycline
      3. Oral Corticosteroid indications
        1. Lupus Pernio
        2. Severe or disfiguring lesions
    3. Neurosarcoidosis: Cranial or Peripheral Neuropathy
      1. First-line: Oral Corticosteroids (e.g. Prednisone)
      2. Other agents
        1. Cyclosporine
        2. Azathioprine
        3. See other management options above under pulmonary Sarcoidosis
  21. Monitoring
    1. Monitoring tools at visits
      1. History and physical
      2. Chest XRay
      3. Spirometry
      4. Specific testing when indicated
    2. Stage I Sarcoidosis
      1. Start with evaluations every 6 months
      2. May space visits to every 12 months if stable
      3. No follow-up if off therapy and stable for 3 years
    3. Stage II to IV Sarcoidosis
      1. Start with evaluations every 3-6 months
      2. Continue visits indefinately
    4. Consultations
      1. Ophthalmology exam annually
  22. Prognosis
    1. Overall mortality: 1-5%
      1. Cause of death in U.S.: Respiratory failure or CHF
    2. Factors suggestive of worse prognosis
      1. Onset after age 40 years
      2. Black race
      3. Chronic Hypercalcemia
      4. Specific higher risk organ involvement
        1. Neurologic involvement
        2. Skin involvement (Lupus Pernio)
        3. Cardiac involvement
        4. Eye involvement (Chronic Uveitis)
        5. Renal involvement (Nephrocalcinosis)
        6. Cystic bone lesions
        7. Involvement of nasal mucosa
        8. Progressive pulmonary fibrosis (advanced radiographic pulmonary staging)
    3. Remission within 2 years
      1. Stage I: 55-90% remission rate
      2. Stage II: 40-70% remission rate
      3. Stage III: 10-20% remission rate
      4. Stage IV: <5% remission rate
  23. Resources
    1. EMedicine: Sarcoidosis
      1. http://emedicine.medscape.com/article/301914-overview#showall
    2. Stop Sarcoidosis Organization
      1. https://www.stopsarcoidosis.org/
  24. References
    1. Klippel (1997) Primer Rheumatic Diseases, AF, p. 325-7
    2. Wilson (1991) Harrison's IM, McGraw-Hill, p.1463-9
    3. Belfer (1998) Am Fam Physician 58(9):2041-50 [PubMed]
    4. Hsu (2001) Am Fam Physician 64(2):289-96 [PubMed]
    5. Soto-Gomez (2016) Am Fam Physician 93(10): 840-8 [PubMed]
    6. Wu (2004) Am Fam Physician 70:312-22 [PubMed]

Chilblain lupus (C0024145)

Concepts Disease or Syndrome (T047)
MSH C535924
SnomedCT 11361009, 72470008, 238928005
Dutch lupus pernio, chillblain lupus erythematodes, lupus; pernio, pernio; lupus
French Lupus pernio, Lupus engelure
German Lupus pernio, Chilblain-Lupus
Italian Lupis pernio, Lupus eritematoso con geloni
Portuguese Lúpus pérnio, Lúpus eritematoso pérnio
Spanish Lupus pernio, lupus pernio, lupus pernio (concepto no activo), Lupus eritematoso en sabañones, sabañón en lupus eritematoso (trastorno), sabañón en lupus eritematoso, sarcoidosis, tipo lupus pernio (trastorno), sarcoidosis, tipo lupus pernio
Japanese 凍瘡状狼瘡, トウソウジョウロウソウ, トウソウジョウエリテマトーデス, 凍瘡状エリテマトーデス
English lupus pernio (diagnosis), lupus pernio, chilblain lupus erythematosus (diagnosis), chilblain lupus erythematosus, Chilblain lupus, chilblain lupus, Lupus pernio, Chilblain lupus erythematosus, Sarcoidosis, lupus pernio type, Chilblain lupus erythematosus (disorder), Sarcoidosis, lupus pernio type (disorder), lupus; pernio, pernio; lupus, Lupus pernio, NOS
Czech Lupus pernio, Chilblain lupus erythematosus
Hungarian Lupus pernio, Fagyásos lupus erythematosus
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Sarcoidosis (C0036202)

Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (CHV) a disease that produces messes especially in the liver, lungs, skin, and lymph nodes
Definition (MEDLINEPLUS)

Sarcoidosis is a disease that leads to inflammation, usually in your lungs, skin, or lymph nodes. It starts as tiny, grain-like lumps, called granulomas. Sarcoidosis can affect any organ in your body.

No one is sure what causes sarcoidosis. It affects men and women of all ages and races. It occurs mostly in people ages 20 to 50, African Americans, especially women, and people of Northern European origin.

Many people have no symptoms. If you have symptoms, they may include

  • Cough
  • Shortness of breath
  • Weight loss
  • Night sweats
  • Fatigue

Tests to diagnose sarcoidosis include chest x-rays, lung function tests, and a biopsy. Not everyone who has the disease needs treatment. If you do, prednisone, a type of steroid, is the main treatment.

NIH: National Heart, Lung, and Blood Institute

Definition (MSHCZE) Onemocnění nejasného původu, předpokládají se imunologické mechanismy při reakci na dosud neznámý antigen či antigeny, postihující řadu orgánů, obv. plíce a nitrohrudní mízní uzliny, popř. mimohrudní orgány (oko, kůže, játra, srdce aj.). Je charakterizována tvorbou granulomů. Probíhá buď subakutně s horečkami, bolestmi kloubů, kožními a plicními příznaky, (srov. Löfgrenův syndrom), nebo chronicky s postupným rozvojem plicního postižení až plicní fibrózy s dušností a omezením plicních funkcí. Bývá rovněž narušen kalciový metabolismus (hyperkalcemie, hyperkalciurie), v séru je možné zjistit zvýšenou aktivitu ACE. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI_NCI-GLOSS) An inflammatory disease marked by the formation of granulomas (small nodules of immune cells) in the lungs, lymph nodes, and other organs. Sarcoidosis may be acute and go away by itself, or it may be chronic and progressive.
Definition (NCI_CDISC) An idiopathic inflammatory disorder characterized by the formation of non-necrotizing epithelioid granulomas which contain giant cells. It usually affects the lungs, lymph nodes, liver, eyes, and skin. Cardiac involvement is also possible.
Definition (NCI) An idiopathic inflammatory disorder characterized by the formation of non-necrotizing epithelioid granulomas which contain giant cells. It usually affects the lungs, lymph nodes, liver, and skin. Cardiac involvement is also possible.
Definition (CSP) inflammatory disease characterized by small lumps or granulomas in lymph nodes and other organs.
Definition (MSH) An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands.
Concepts Disease or Syndrome (T047)
MSH D012507
ICD9 135
ICD10 D86, D86.9, D80-D89
SnomedCT 31541009, 154425004
English Besnier-Boeck Disease, Boeck's Sarcoid, Disease, Schaumann, Sarcoid, Boeck's, Sarcoidoses, Sarcoidosis, Schaumann Disease, Besnier Boeck Disease, Boecks Sarcoid, SARCOIDOSIS, Boecks sarcoidosis, Sarcoidosis, unspecified, Boeck's sarcoidosis, SCHAUMANN DIS, BESNIER BOECK DIS, lymphogranulomatosis (benign), Besnier Boeck disease, sarcoidosis (diagnosis), sarcoidosis, benign lymphogranulomatosis, benign lymphogranulomatosis (diagnosis), benign lymphogranulomatosis of Schaumann, Sarcoid, Sarcoidosis NOS, Besnier-Boeck-Schaumann Syndrome, Syndrome, Besnier-Boeck-Schaumann, Syndrome, Schaumann, Schaumann's Syndromes, Boeck's Disease, Besnier Boeck Schaumann Syndrome, Boecks Disease, Syndrome, Schaumann's, Schaumann's Syndrome, Boeck Disease, Schaumann Syndrome, Sarcoidosis [Disease/Finding], boeck's disease, sarcoids, schaumann disease, schaumann syndrome, boeck's sarcoid, schaumann's disease, Besnier-Boeck-Schaumann's disease, Boeck's disease, Schaumann's disease, sarcoid, Besnier-Boeck-Schaumann syndrome, Boeck's sarcoid, Benign lymphogranulomatosis of Schaumann, Darier-Roussy sarcoid, Lupus pernio of Besnier, Miliary lupoid of Boeck, Sarcoidosis (disorder), lymphogranulomatosis, benign; lymphogranulomatosis, Hutchinson-Boeck, Schaumann; benign lymphogranulomatosis, Schaumann; disease or syndrome, Besnier-Boeck, Besnier; lupus pernio, Boeck; disease, Boeck; sarcoid, lymphogranulomatosis; benign, sarcoid; Boeck, sarcoid; Darier-Roussy, syndrome; Schaumann, Darier-Roussy; sarcoid, Sarcoidosis, NOS, Boeck Sarcoid, Besnier-Boeck-Schaumann disease, Boeck Sarcoid, any site, Benign Lymphogranulomatosis, Benign Lymphogranulomatosis, Schaumann's, Lupus pernio, Besnier, Miliary Lupoid of Boeck, Darier-Roussy Sarcoid, Sarcoid NOS
French SARCOIDOSE, Sarcoïde, Maladie de Schaumann, Sarcoïdose SAI, Maladie de Boeck, Lymphogranulomatose bénigne, Sarcoïdose, Maladie de Besnier-Boeck-Schaumann
Portuguese SARCOIDOSE, Doença de Besnier-Boeck-Schaumann, Sarcóide, Sarcoidose NE, Síndrome de Schaumann, Doença de Boeck, Sarcoide de Boeck, Doença de Besnier-Boeck, Sarcoidose
Spanish SARCOIDOSIS, Enfermedad de Schaumann, Enfermedad de Besnier-Boeck-Schaumann, Enfermedad de Boeck, Sarcoidosis NEOM, Sarcoide, linfogranulomatosis benigna de Schaumann, lupoide miliar de Boeck, lupus pernio de Besnier, sarcoide de Boeck, sarcoide de Darier - Roussy, sarcoidosis (trastorno), sarcoidosis, síndrome de Besnier - Boeck - Schaumann, Sarcoidosis, Enfermedad de Besnier-Boeck, Sarcoide de Boeck
German SARKOIDOSE, Besnier-Boeck-Schaumann-Krankheit, Sarkoid, Sarkoidose NNB, Boeck-Krankheit, Sarkoidose, nicht naeher bezeichnet, Besnier-Boeck-Schaumann-Syndrom, Boeck-Sarkoid, Morbus Schaumann, Sarkoidose, Schaumann-Krankheit
Dutch ziekte van Schaumann, ziekte van Besnier-Boeck-Schaumann, ziekte van Boeck, sarcoïd, sarcoïdose NAO, Besnier; lupus pernio, Boeck; sarcoïd, Boeck; ziekte, Darier-Roussy; sarcoïd, Schaumann; benigne lymfogranulomatose, Schaumann; ziekte of syndroom, benigne; lymfogranulomatose, lymfogranulomatose; benigne, sarcoïd; Boeck, sarcoïd; Darier-Roussy, syndroom; Schaumann, Sarcoïdose, niet gespecificeerd, sarcoidose, Besnier-Boeck-ziekte, Schaumann-ziekte, Ziekte van Besnier-Boeck, Boeck-sarcoïd, Sarcoïdose
Italian Sarcoide, Malattia di Boeck, Sarcoidosi NAS, Malattia di Schaumann, Malattia di Besnier-Boeck, Malattia di Besnier-Boeck-Schaumann, Sarcoide di Boeck, Sarcoidosi
Japanese サルコイド, ベニエー・ベック・シャウマン病, シャウマン病, サルコイドーシスNOS, サルコイドーシス, サルコイド, ベニエーベックシャウマンビョウ, ベックビョウ, サルコイドーシスNOS, シャウマンビョウ, ベニエー・ベック・シャウマン病, Boeckサルコイド, サルコイドーシス, Schaumann病, サルコイド症, ベック病, ベック類肉腫, ベニエー-ベック病, 良性リンパ肉芽腫, 良性リンパ肉芽腫症, 血管類狼瘡, 類肉腫, 類肉腫症, ザルコイドーシス, ベズニエ-ベック-シャウマン症候群, ベックサルコイド
Swedish Sarkoidos
Czech sarkoidóza, Sarkoidóza NOS, Boeckova nemoc, Schaumannova nemoc, Sarkoidóza, Sarkoid, Boeckova sarkoidóza, Besnier-Boeck-Schaumannova nemoc, Besnier-Boeckova nemoc, benigní lymfogranulomatóza, Besnierova-Boeckova-Schaumannova choroba
Finnish Sarkoidoosi
Russian BEN'E-BEKA BOLEZN', BEKA SARKOID, SARKOIDOZ, SHAUMANNA BOLEZN', БЕКА САРКОИД, БЕНЬЕ-БЕКА БОЛЕЗНЬ, САРКОИДОЗ, ШАУМАННА БОЛЕЗНЬ
Korean 상세불명의 사르코이도시스, 사르코이도시스
Croatian SARKOIDOZA
Polish Sarkoidoza
Hungarian Sarcoidosis, Schaumann-betegség, Sarcoidosis k.m.n., Besnier-Boeck-Schaumann-betegség, Boeck-betegség, Sarcoid
Norwegian Sarkoidose, Morbus Boeck, Boecks sykdom, Boecks sarkoid
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Uveoparotid Fever (C0042171)

Definition (MSH) A manifestation of sarcoidosis marked by chronic inflammation of the parotid gland and the uvea.
Concepts Disease or Syndrome (T047)
MSH D014608
ICD10 D86.89
SnomedCT 4416007, 31541009
English Fevers, Uveoparotid, Uveoparotid Fever, Uveoparotid Fevers, Fever, Uveoparotid, Heerfordt-Waldenstroem syndr, Heerfordt Syndrome, Syndrome, Heerfordt, Uveoparotitides, Uveoparotitis, uveoparotid fever (diagnosis), uveoparotid fever, Heerfordt syndrome, Uveoparotid fever [Heerfordt], Uveoparotid Fever [Disease/Finding], heerfordts syndrome, uveoparotitis, heerfordt syndrome, heerfordt's syndrome, Uveoparotid fever, Heerfordt's syndrome, Heerfordt-Waldenstroem syndrome, Heerfordt's syndrome (disorder), Heerfordt, febris; uveoparotid, fever; uveoparotid, uveoparotid; febris, uveoparotid; fever
Swedish Uveoparotid feber
Japanese ヘールフォルトショウコウグン, Heerfordt症候群, ブドウ膜耳下腺熱, Heerfordt病, ぶどう膜耳下腺炎, ぶどう膜耳下腺熱, ヘールフォルト病, ヘールフォルト症候群, 葡萄膜耳下腺熱
Czech uveoparotická horečka, Heerfordtův syndom
Finnish Uveoparotiitti
Russian KHEERFORDTA SINDROM, UVEOPAROTIDNAIA LIKHORADKA, UVEOPAROTIT, УВЕОПАРОТИДНАЯ ЛИХОРАДКА, УВЕОПАРОТИТ, ХЕЕРФОРДТА СИНДРОМ
Italian Sindrome di Heerfordt, Uveoparotite, Febbre con uveite e tumefazione della parotide
Polish Zespół Heerfordta
Hungarian Heerfordt-syndroma
Norwegian Uveoparotid feber, Febris uveoparotidea
Portuguese Doença de Heerfordt, Uveoparotite, Febre Uveoparotídea, Síndrome de Heerfordt, Uveoparotidite
Dutch febris; uveoparotidea, koorts; uveoparotidea, uveoparotidea; febris, uveoparotidea; koorts, syndroom van Heerfordt, Febris uveoparotidea, Heerfordt, syndroom van, Parotitis, uveo-, Uveoparotitis
Spanish fiebre uveoparotídea, síndrome de Heerfordt (trastorno), síndrome de Heerfordt, Fiebre Uveoparotidea, Síndrome de Heerfordt, Uveoparotitis
French Fièvre uvéoparotidienne, Uvéoparotidite, Syndrome de Heerfordt
German Febris uveoparotidea, Heerfordt-Syndrom, Uveoparotitis
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Lofgrens syndrome (C0340164)

Concepts Disease or Syndrome (T047)
SnomedCT 9614001, 181225007, 238676008
English BHL sarcoidosis, eryth nodosum, Erythema nodosum, BHL sarcoid, Bilateral hilar adenopathy syndrome, Lofgren's syndrome, Bilateral hilar lymph node sarcoidosis with erythema nodosum, Erythema nodosum with bilateral hilar lymph node sarcoidosis, Bilateral hilar adenopathy syndrome (disorder), Bilateral hilar adenopathy syndrome (disorder) [Ambiguous], Loefgren syndrome, Loefgrens syndrome, Lofgrens syndrome (disorder), Lofgrens syndrome, Loefgrens syndrome (disorder)
Dutch Löfgren-syndroom
French Syndrome de Loefgren
German Loefgren-Syndrom
Italian Sindrome di Loefgren
Portuguese Síndrome de Loefgren
Spanish Síndrome de Loefgren, síndrome de Loefgrens (trastorno), síndrome de Lofgren, síndrome de adenopatía hiliar bilateral (trastorno), síndrome de adenopatía hiliar bilateral, eritema nodoso con adenopatías perihiliares bilaterales por sarcoidosis, síndrome de adenopatía hilar bilateral (trastorno), síndrome de adenopatía hilar bilateral, síndrome de adenopatía hiliar bilateral (concepto no activo), síndrome de Loefgrens, síndrome de Lofgrens, sarcoidosis bilateral de ganglios linfáticos hiliares con eritema nudoso, síndrome de Lofgrens (trastorno)
Japanese レフグレン症候群, レフグレンショウコウグン
Czech Lofgrenův syndrom
Hungarian Loefgren-syndroma
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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