Epidemiology
- Prevalence: varies from 0.04/100 K in Spain to 64/100 K in Sweden
- Sex: slight F>M
- Race: American blacks have 10-17x incidence that of whites
- Age of onset: most commonly between 20-40 y/o (can occur in children and elderly: elderly are usually chronic cases)
- Tobacco: higher incidence of Sarcoid in non-smokers (compared to smokers)
- Genetics: slightly increased (possibly due to low penetrance) incidence in twins (monozygotic > dizygotic) and other family members (including spouses)
- Increased incidence in blacks with HLA-Bw 15
- Alleles which increase risk of disease: HLA DR 11, 12, 14, 15, and 17
- Alleles which are protective: HLA DR1, DR4, and possibly HLA DQ 0202
- Resolution of disease is linked to HLA-B8 (this antigen also occurs more commonly in patients with sarcoid arthritis and E. nodosum)
Physiology
-
Immunologic Disease of Unknown Etiology
-
T-cell alveolitis: initial process (BAL shows increased CD4 cells and increased amount of alveolar macrophages)
- Decreased peripheral blood CD4: CD8 ratio
- Granuloma formation (found in most organs): alveolar macrophages are incorporated into granulomas (alveolar macs take up Gallium when scanned)
- Even early in disease, granulomas can be found in lungs and liver [Hunninghake; Sarc Vasc Diff Lung Dis, 1999]
- PCR has detected Mycobacteria Tuberculosis in tissue from 7/16 patients in one series
- Upper Airway Involvement: laryngeal/ posterior pharyngeal/ vocal cord infiltration by granulomas
- Lower Airway Involvement: granulomatous involvement of trachea/ bronchi (or stenosis due to scarring associated with disease)
- Extrinsic compression of bronchus by adenopathy is rare in Sarcoidosis
Pathology
- Non-caseating granulomas (typically along lymphatic routes)
- Whorls of elongated fibroblasts/ epithelioid cells with mononuclear cells
- Extensive necrosis is rare
- Giant cells (may be either Langhan or foreign-body type) with multinucleated crystalline or cellular inclusions (inclusions: Asteroid bodies, Schaumann bodies, or Hamasaki-Wesenberg bodies)
- Similar granulomas may also be seen in Berylliosis/ certain malignancies/ immune deficiencies/ Foreign Body Granulomatosis
- Nodular/Necrotizing Sarcoid: confluent granulomas/necrotic granulomatous vasculitis of pulmonary arteries, veins
Diagnosis
Arterial Blood Gas (ABG)/Oximetry
- Exercise-Related Desaturation: due mainly to diffusion defect
- Exercise pO2 correlates with right ventricular ejection fraction
Serum Chemistry
- Hypercalcemia (see Hypercalcemia, [[Hypercalcemia]])
Purified Protein Derivative (PPD)
- Usually Partial or Complete Anergy: PPD is negative in >66% of patients
- Sometimes positive PPD seen initially or with remission
- Previously positive PPD may revert to negative during active disease, returning to positive with cure of sarcoidosis
- Strongly positive PPD is rare in sarcoidosis
Kveim Test
- Positive: although not currently used
Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests, [[Pulmonary Function Tests]])
- No correlation of PFT’s with CXR Findings, Disease Activity, and Disease Reversibility
- Radiographic Stage 1: PFT’s are abnormal in 20% of cases
- Radiographic Stage 2-3: PFT’s are abnormal in 40-70% of cases
- Spirometry: obstruction is observed in 30% of cases
- Lung Volumes
- TLC: correlates with RV-EF
- VC: decreased
- DLCO: decreased early (due to interstitial and pulmonary vascular disease)
Bronchoscopy (see Bronchoscopy, [[Bronchoscopy]])
- Appearance of Airways: granular “cobble-stoning” of airways
- Bronchoalveolar Lavage (BAL): lymphocytosis (>35%) may be seen
- High CD4/CD8 ratio >3.5 (ratio >5 is highly suggestive of sarcoidosis)
- Especially high in Lofgren syndrome patients
- Previous studies suggest that BAL lymphocytosis >28% predicts clinical deterioration: however, this is controversial
- Neutrophilic predominance: may correlate with presence of end-stage pulmonary fibrosis
- High CD4/CD8 ratio >3.5 (ratio >5 is highly suggestive of sarcoidosis)
- Endobronchial Biopsy (EBB): indicated for gross airway findings
- Trans-Bronchial Biopsy (TBB)
- Positive in 50-60% of cases with normal CXR
- Positive in 85-90% with abnormal CXR
CXR/Chest CT Pattern
- Usually, adenopathy precedes parenchymal disease (rare for adenopathy/parenchymal disease to recur after spontaneous clearing)
- CXR Patterns
- Hilar Lymphadenopathy
- Interstitial Lung Disease
- Bullous-Cystic Disease
- Lung Nodules: may cavitate
- Radiographic Staging: however, disease does not progress temporarily through these “stages”
- Stage 0 (5-10% of cases): normal
- In these cases, HRCT may demonstrate parenchymal abnormalities and adenopathy
- Stage 1: bilateral hilar adenopathy
- Stage 2a: bilateral hilar adenopathy + diffuse lung disease
- Stage 2b: diffuse lung disease without hilar adenopathy
- Stage 3: fibrosis + distortion of lung parenchyma
- Stage 0 (5-10% of cases): normal
High-Resolution Chest CT (HRCT)
- May show nodules along bronchovascular bundles, particularly in mid-upper lung fields (”beading”), pleural or subpleural nodules, septal lines, confluent opacities with air bronchograms, cystic or bronchiectatic lucencies, honeycombing, or bullae
Bone Scan
- xxx
Gallium Scan
- “Panda Pattern”: uptake in hilar and mediastinal nodes, lacrimal glands, and salivary glands
- Non-specific
- May guide biopsy site
- Does not reliably predict prognosis or responsiveness to therapy
- May aid in monitoring of disease
Cardiac MRI
- Useful to Evaluate Cardiac Sarcoid
Brain MRI
- Useful to Evaluate Neurosarcoid
Serology/Inflammatory Markers
- ANA/RF/Anti-Lymphocyte Ab: may be positive
- Circulating Immune Complexes: may be seen (increased levels may occur with symptoms)
- Polyclonal Hyperglobulinemia/Macroglobulinemia: may be present
- Lysozyme/Thermolysin-Like Metallopeptidase: elevated
Serum Angiotensin Converting Enzyme (ACE) Level (see Serum Angiotensin Converting Enzyme, [[Serum Angiotensin Converting Enzyme]])
- Elevated >2 Standard Deviations in 41-80% of Sarcoidosis Cases
- However, ACE level may also be elevated in HIV, hepatitis, miliary TB, Gaucher’s, Histoplasmosis
- May be decreased with corticosteroid or ACE inhibitor use
- May Be Useful to Monitor Disease Activity
Biopsy
- Liver/Lymph Node Biopsy: high Incidence of false-positives
- Skin/Conjunctival Biopsy: low sensitivity, but high specificity
- Nasal Mucosal/Muscle/Spleen/Lacrimal Gland/Salivary Gland Biopsy: may be useful in some cases
Clinical Manifestations
Cardiovascular Manifestations
General Comments
- General Comments: cardiac involvement may occur in the absence of other organ involvement
Conduction Defects (see Heart Blocks, [[Heart Blocks]])
- Epidemiology
- Clinical
- Syncope (see Syncope, [[Syncope]])
Arrhythmias
- Epidemiology
- Clinical
- Premature Ventricular Contractions (PVC’s)/Ventricular Tachycardia (VT) (see Ventricular Tachycardia, [[Ventricular Tachycardia]])
- Syncope (see Syncope, [[Syncope]])
Restrictive Cardiomyopathy
- Epidemiology:
Pericardial Effusion/Tamponade (see Pericardial Effusion, [[Pericardial Effusion]] and Tamponade, [[Tamponade]])
- Epidemiology: uncommon
Constrictive Pericarditis (see Constrictive Pericarditis, [[Constrictive Pericarditis]])
- Epidemiology: uncommon
Pulmonary Hypertension with Cor Pulmonale (see Pulmonary Hypertension, [[Pulmonary Hypertension]])
- Epidemiology:
Superior Vena Cava (SVC) Syndrome (see Superior Vena Cava Syndrome, [[Superior Vena Cava Syndrome]])
- Epidemiology: uncommon
- Physiology: due to mediastinal lymphadenopathy
Substernal Chest Pain (see Chest Pain, [[Chest Pain]])
- Epidemiology: common
Constitutional Manifestations
- Fever (see Fever, [[Fever]])
Dermatologic Manifestations
- Erythema Nodosum (see Erythema Nodosum, [[Erythema Nodosum]]): painful, nodular panniculitis, and vasculitis
- Subcutaneous Nodules: on extremities
- Nodular or Flat Plaques/Papules
- Lupus Pernio: bluish-purplish elevations
- Loffgren’s Syndrome
- Arthralgias
- Bihilar Lymphadenopathy
- Erythema Nodosum
Endocrine Manifestations
- Parotid Gland Enlargement
- SIADH/DI/primary polydipsia
- Hypercalcemia (see Hypercalcemia, [[Hypercalcemia]]): due to increased calcium absorption, enhanced sensitivity to vit. D, or increased vit. D metabolite production by granulomas)
- Hypercalciuria
- PTH may be decreased (due to hypercalcemia)
- Involvement of pituitary/ thyroid/ parathyroid/ adrenal glands/ testes/ epididymis/ uterus (rare): no direct effect on fertility
- Pregnancy: disease probably improves during pregnancy, with disease exacerbation post-partum
Gastrointestinal Manifestations
- Anorexia (see Anorexia, [[Anorexia]])
- Appendiceal Involvement: pain may mimic appendicitis
- Dysphagia Due to Infiltration of Esophageal Wall and/or Esophageal Compression by Lymphadenopathy (see Dysphagia, [[Dysphagia]])
- Esophageal Achalasia (see Esophageal Achalasia, [[Esophageal Achalasia]]): due to involvement of Auerbach’s plexus
- Gall Bladder Involvement: pain may mimic cholecystitis
- Malabsorption: due to jejunal atrophy/non-necrotizing granuloma of stomach, small intestine, colon
- Pancreatic Involvement: pain may mimic pancreatitis
- Weight Loss (see Weight Loss, [[Weight Loss]])
Hematologic Manifestations
- Cervical/Supraclavicular/Epitrochlear Lymphadenopathy (see Lymphadenopathy, [[Lymphadenopathy]]): usually small, discrete, mobile nodes
- Hypersplenism (see Splenomegaly, [[Splenomegaly]]): marked splenomegaly occurs more often with chronic disease/ may produce pancytopenia
- Thrombocytopenia (see Thrombocytopenia, [[Thrombocytopenia]]): may occur without splenomegaly
Hepatic Manifestations
- General Comments: usually asymptomatic
- Hepatic Parenchymal Lesions (see Hepatic Mass, [[Hepatic Mass]])
- RUQ Pain
- Jaundice
- Hepatomegaly (see Hepatomegaly, [[Hepatomegaly]])
- Portal fibrosis
- Cirrhosis (see End-Stage Liver Disease, [[End-Stage Liver Disease]]): rare
- Hepatitis: rare
- Portal Hypertension (see Portal Hypertension, [[Portal Hypertension]]): due to damaged hepatic veins
Neurologic Manifestations (5% of cases)
- Cranial Nerve Palsies (most common presentation): usually facial nerve (CN-7)
- Heerfordt’s syndrome (Uveoparotid fever): CN-7 palsy+ parotid involvement + uveitis
- Fatigue (see Fatigue, [[Fatigue]])
- Hearing Loss/Vestibular Dysfunction (due to neuropathy or vasculitis of cranial nerve)
- Peripheral Neuropathy (see xxxx, [[xxxx]]): parasthesias
- Meningitis (see Meningitis, [[Meningitis]]): CSF may be normal (or may demonstrate increased protein, pleocytosis, hypoglycorrhachia, elevated CSF pressure)
- Manifestations of Space-Occupying Lesions
- Headache (see Headache, [[Headache]])
- Seizures (see Seizures, [[Seizures]])
- Obtundation-Coma/Encephalopathy (see Obtundation-Coma, [[Obtundation-Coma]])
- Cerebellar Dysfunction (se xxxx, [[xxxx]])
- Spinal Cord Compression (see xxxx, [[xxxx]])
- Brainstem Dysfunction: due to brainstem lesions
- Acute Hypoventilation (see Acute Hypoventilation, [[Acute Hypoventilation]])
- Chronic Hypoventilation (see Chronic Hypoventilation, [[Chronic Hypoventilation]])
Ophthalmologic Manifestations
- Uveitis: most common ophthalmologic manifestation of sarcoidosis
- Anterior Uveitis: inflammation in the portion of the uveal tract extending forward from the iris and ciliary body -> asymptomatic or red painful eye with photophobia
- Exam: “mutton fat” in anterior chamber and/or posterior cornea
- Intermediate Uveitis: inflammation in the vitreous, pars plana, or peripheral retina -> floaters or other visual disturbances
- Posterior Uveitis: involves central portions of the retina (with venulitis that causes exudation of protein out of the retinal veins) -> retinal scarring and permanent vision loss
- Fluorescence Angiogram: leaking retinal veins, leads to appearance of candle wax drippings
- Anterior Uveitis: inflammation in the portion of the uveal tract extending forward from the iris and ciliary body -> asymptomatic or red painful eye with photophobia
- Optic Neuritis (rare): usually presents with sudden loss of vision or color vision
- Exam: papillitis, papilledema, neovascularization with optic atrophy
- Vitreous Opacities/Choroiditis/Conjunctival Granulomas: photophobia, eye pain, visual loss
- Lacrimal Gland Enlargement (25% of cases): may produce sicca-like syndrome
- Biopsy of Lacrimal Gland: may be useful
- Exophthalmos: due to orbital disease
Otolaryngologic Manifestations
Nasal Polyps (see Nasal Polyps, [[Nasal Polyps]])
- Clinical: nasal congestion
Laryngeal/Posterior Pharyngeal/Vocal Cord Granulomas
- Clinical
- Hoarseness (see Hoarseness, [[Hoarseness]])
- Upper Airway Obstruction (see Obstructive Lung Disease, [[Obstructive Lung Disease]]): laryngeal granulomas seen mostly with lupus pernio, but occur with other dermatologic manifestations
Pulmonary Manifestations
General Comments
- Predominant Anatomic Sites of Pulmonary Involvement
- Bronchovascular Bundles: lesions along airways are common (and lesions involving the pulmonary vasculature may explain the occasional development of pulmonary hypertension)
- Perilymphatic Areas: subpleural lesions are common
- Distribution: upper lobe-predominant
Alveolar Sarcoidosis (see Pneumonia, [[Pneumonia]])
- Diagnosis
- CXR/Chest CT
- Fluffy alveolar densities
- “Reverse pulmonary edema” peripheral infiltrates, resembling chronic eosinophilic pneumonia or COP, have been reported
- “Fairy Ring”/”Atoll Sign”/”Reverse Halo Sign”: normal or ground-glass region of parenchyma surrounded by dense ring of alveolar consolidation
- CXR/Chest CT
Bullous-Cystic Sarcoid (see Cystic-Cavitary Lung Lesions, [[Cystic-Cavitary Lung Lesions]])
- Clinical
- Cavities may become colonized with Aspergillus
- Severe bullous disease may cause the “Vanishing Lung Syndrome”
- Treatment/Prognosis: cavities may resolve with or without therapy
Hilar/Mediastinal Lymphadenopathy (see Mediastinal Mass, [[Mediastinal Mass]])
- Epidemiology: occurs in 75-90% of cases
- Diagnosis
- CXR/Chest CT
- Usually bilateral and symmetric
- Involves hilar, paratracheal, subcarinal, and other mediastinal lymph nodes
- Hilar Nodes: “eggshell” calcification may occur
- Subcarinal/Other Mediastinal Nodes: non-specific calcification may occur with long-standing disease
- Posterior Mediastinal Mass: may occur
- CXR/Chest CT
- Clinical: substernal chest pain
- Treatment/Prognosis: may regress, remain stable for years, or grow slowly
Nodular/Necrotizing Sarcoidosis (see Lung Nodule or Mass, [[Lung Nodule or Mass]] and Cystic-Cavitary Lung Lesions, [[Cystic-Cavitary Lung Lesions]])
- Epidemiology: first described in 1973 by Liebow
- Rare: only 3 of 150 cases in one series
- Sex: female predominance
- Physiology: granulomatous vasculitis -> necrosis may develop within the granulomatous lesion
- Clinical: may be asymptomatic
- Chest Pain
- Cough
- Dyspnea
- Fatigure/Malaise
- Fever
- Hemoptysis
- Ocular or Central Nervous System Involvement
- Weight Loss
- Diagnosis
- Chest CT: confluent or diffuse >1 cm solitary or multiple lung nodules (possibly associated with lymphadenopathy)
- Nodules may cavitate (calcification is usually absent): cavitation (and granulomatous vasculitis or pulmonary arteries and veins/necrosis of nodules) clinically distinguishes nodular/necrotizing sarcoidosis from typical sarcoidosis
- No lobar predilection
- Predominantly located subpleural and along bronchovascular bundles (similar to other Sarcoid lesions)
- Chest CT: confluent or diffuse >1 cm solitary or multiple lung nodules (possibly associated with lymphadenopathy)
- Treatment/Prognosis
- Usually benign clinical course with or without corticosteroids
- Some cases have severe pulmonary or extrapulmonary involvement
- Few cases develop pulmonary hypertension due to pulmonary vasculitis
Pleural Involvement
- Epidemiology: uncommon
- Clinical
- Chylothorax (see Pleural Effusion-Chylothorax, [[Pleural Effusion-Chylothorax]]): only 2 reported cases
- Pleural Effusion (see Pleural Effusion-Exudate, [[Pleural Effusion-Exudate]]): occur in <3% of cases
- Small and May be Bilateral
- Lymphocyte-Predominant: may be >80%
- Exudate (usually): by total protein criteria alone (since pleural LDH is usually normal)
- Pleural Thickening
- Pneumothorax (see Pneumothorax, [[Pneumothorax]])
- Most cases are associated with fibrocystic disease
- May be recurrent
Pulmonary Hypertension (see Pulmonary Hypertension, [[Pulmonary Hypertension]])
- Epidemiology: occurs in 1-28% of cases
- Physiology
- Likely due to destruction of the capillary bed by fibrosis and/or resultant chronic hypoxemia: however, the severity of pulmonary hypertension does not correlate well with the degree of parenchymal lung disease and blood gas abnormalities
- Other potential mechanisms: extrinsic compression of large pulmonary arteries by mediastinal and hilar adenopathy, and direct granulomatous infiltration of the pulmonary vasculature (especially the pulmonary veins, which sometimes mimic pulmonary veno-occlusive disease)
- More rarely, portopulmonary hypertension secondary to hepatic sarcoidosis can occur
Pulmonary Veno-Occlusive Disease (VOD) (see Pulmonary Veno-Occlusive Disease, [[Pulmonary Veno-Occlusive Disease]])
- Epidemiology: few case reports
Reticulonodular Interstitial Lung Disease (see Interstitial Lung Disease-Etiology, [[Interstitial Lung Disease-Etiology]])
- Epidemiology: most common CXR pattern in sarcoidosis
- Diagnosis
- HRCT (superior to normal CT in assessing fine parenchymal abnormalities and separating alveolitis from fibrosis): lymphadenopathy/focal nodular (usually miliary <3 mm micronodules/ may be up to 3-5 mm nodules) or ground-glass opacities (peribronchovascular distribution/predilection for upper, middle, posterior lung zones)
- Patchy nodules or segmental ground glass opacities without distortion are characteristic of early alveolitis (with minimal pulmonary dysfunction)
- HRCT scores do not correlate with the Gallium and BAL scores of disease activity
- HRCT (superior to normal CT in assessing fine parenchymal abnormalities and separating alveolitis from fibrosis): lymphadenopathy/focal nodular (usually miliary <3 mm micronodules/ may be up to 3-5 mm nodules) or ground-glass opacities (peribronchovascular distribution/predilection for upper, middle, posterior lung zones)
Rounded Atelectasis (see Rounded Atelectasis, [[Rounded Atelectasis]])
- Epidemiology: case reports
Tracheobronchial Airway Obstruction (see Obstructive Lung Disease, [[Obstructive Lung Disease]])
- Physiology: granulomatosus tracheobronchial involvement
- May progress to stenotic tracheobronchial scarring
- Diagnosis
- CXR/Chest CT: right middle lobe involvement is uncommon
- PFT’s: obstruction
- Bronchoscopy: characteristic diffuse granular bronchial mucosa (“cobble-stoning”), obstructing lesions, stenoses
- Clinical: may present as either large or small airway obstruction
- Bronchiectasis (see Bronchiectasis, [[Bronchiectasis]])
- Dyspnea (see Dyspnea, [[Dyspnea]])
- Paroxysmal Cough (see Cough, [[Cough]])
- Post-Obstructive Pneumonia (see Pneumonia, [[Pneumonia]])
- Wheezing (see Obstructive Lung Disease, [[Obstructive Lung Disease]])
Renal Manifestations
- Chronic Kidney Disease (CKD) (see Chronic Kidney Disease, [[Chronic Kidney Disease]]): due to renal granulomas, calculi (due to hypercalcemia/hypercalcuria)
Rheumatologic Manifestations
- Polyarthritis/Monoarthritis (see Arthritis, [[Arthritis]]): usually associated with Loffgren’s syndrome
- Predominant Anatomic Sites of Involvement
- Ankles
- Wrists
- Predominant Anatomic Sites of Involvement
- Myopathy (see Myopathy, [[Myopathy]]): muscle granulomas are usually symptomatic (may occasionally produce acute myopathy with marked CK elevation)
- Lytic or Sclerotic Bone Lesions: usually associated with skin or visceral disease
- Predominant Anatomic Sites of Involvement
- Tubular hand bones: 3% of cases
- Thoracic spine
- Ribs
- Sternum
- Gallium Scan: increased uptake in “Panda” pattern
- Bone Scan
- Predominant Anatomic Sites of Involvement
- Asymmetric Pseudoclubbing: due to bone cysts with dactylitis
Treatment
Corticosteroids (see Corticosteroids, [[Corticosteroids]])
- Dose: prednisone 20-40 mg/day x 6-12 weeks, taper over months to lowest effective dose, continue for months-years
- Indicated for eye, heart, brain, kidney, lung disease, or persistent hypercalcemia
- Discontinued due to SE in 5-10% of cases
Chloroquine (see Chloroquine, [[Chloroquine]])
- Dose: chloroquine 250 mg PO BID x 3 months, then 250 mg PO qday x 3 months
- Useful for chronic pulmonary and cutaneous disease (particularly Lupus Pernio)
- Decreases hypercalcemia [Annals, 1989]
- May be used as steroid-sparing agent
- Requires q3 month retinal exam to detect retinopathy
- SE: reversible myopathy or neuropathy
Hydroxychloroquine (see Hydroxychloroquine, [[Hydroxychloroquine]])
- Dose: plaquenil 200 mg qday-BID PO qday x 3-6 months
- Useful for skin lesions, hypercalcemia, and neurosarcoid
- Safer and almost as effective as Chloroquine (has replaced Chloroquine today)
- Requires q3 month retinal exam to detect retinopathy (although retinopathy has not been reported)
- SE: decreases blood glucose (may be useful in cases with co-existent DM)/ reversible myopathy or neuropathy
Quinacrine (see Quinacrine, [[Quinacrine]])
- Effective in cutaneous disease
- SE: ocular toxicity, psychosis
Methotrexate (see Methotrexate, [[Methotrexate]])
- Dose: methotrexate 10-15 mg PO q week x 3-6 months or to disease activity
- Steroid-sparing effect/ improvement in lung function, skin lesions, and lung disease by MRI in non-randomized trials/ particularly effective for musculoskeletal manifestations
- SE: hepatic fibrosis is rare in low-dose- treated cases
Indomethacin (see Indomethacin, [[Indomethacin]])
- May be used in acute sarcoidosis (with uveitis, polyarthritis, E. nodosum)
Azathioprine (Imuran) (see Azathioprine, [[Azathioprine]])
- Dose: azathioprine 150 mg PO qday x 3-6 months
- Effective in small subset of steroid and chloroquine-unresponsive cases (long-term effects are unknown)
- SE: immunosuppression
Chlorambucil (see Chlorambucil, [[Chlorambucil]])
- Dose: chlorambucil 5 mg PO qday x 3 months
- May be useful in steroid-unresponsive cases
- SE: immunosuppression, GI symptoms, azoospermia, amenorrhea, pulmonary fibrosis, hepatitis, seizures
Cyclophosphamide (Cytoxan) (see Cyclophosphamide, [[Cyclophosphamide]])
- Dose: cyclophosphamide 50 mg PO qday x 3 weeks
- SE: immunosuppression
Oxyphenbutazone (see Oxyphenbutazone, [[Oxyphenbutazone]])
- Dose: oxyphenbutazone 400 mg PO qday x 3-6 months
- Effective in placebo-controlled trial in Lancet, 1967 (50% improvement in steroid and oxy group)/ improvement most evident in patients treated within 2 years of disease onset
- SE: PUD
Thalidomide (see Thalidomide, [[Thalidomide]])
- Dose: thalidomide 200 mg PO qday x 2 weeks, then 100 mg PO qday x 11 weeks
- May be effective in steroid-unresponsive cases with skin and lung disease
Etanercept/Infliximab/Pentoxifylline/Cellcept (see xxxx, [[xxxx]])
- Useful in patients with poor steroid response or severe steroid side effects
Cyclosporine A (see Cyclosporine A, [[Cyclosporine A]])
- Effective in neurosarcoidosis, patients refractory to steroids (Neurosarcoid, 1992)
Tranilest (see Tranilest, [[Tranilest]])
- An anti-allergic agent, may have some anti-fibrotic effects
Allopurinol (see Allopurinol, [[Allopurinol]])
- Can induce regression in cutaneous and pulmonary sarcoidosis
Radiation Therapy
- Formerly used to treat hilar and mediastinal adenopathy
- Whole brain irradiation has been used to control meningeal and neurosarcoid (one case with thalamic, posterior third ventricle granuloma showed improvement)
- Megavoltage XRT has been used to treat laryngeal Sarcoid refractory to steroids
Lung, Heart, and Liver Transplantation (see xxxx, [[xxxx]])
- Have been successfully performed in sarcoidosis (granulomas may recur in transplanted organs)
- 2 cases of donor-organ sarcoidosis led to active sarcoidosis in organ recipient
Monitors of Sarcoid Disease Activity
- ACE level: decreasing level correlates with remission and response to steroids
- Gallium scan: may be useful in some settings
- Lung DTPA: increased clearance correlates with deterioration of lung function
- BAL CD4/CD8 ratio: persistent elevation for >12 months predicts a greater probability of non-remission or deterioration
Prognosis
- Course: 30-50% remit (within 3 years)/30% progress/20-30% remain stable (over 5-10 years)
References
Pulmonary Hypertension
- Pulmonary haemodynamics at rest and during exercise in patients with sarcoidosis. Respiration 1984;46:26–32
- Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur Respir J 2005;25:783–8
- Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J 2008;32:296–302
- Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 2006;61:68–74
- Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 2006;129:1246 –52
- The Clinical Features of Sarcoidosis: A Comprehensive Review. Clin Rev Allergy Immunol. 2014 Oct 2 (Epub ahead of print) [MEDLINE]