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![Defn
Sarcoidosis is a multisystem granulomatous disorder of
unknown aetiology that is characterised by the presence of
non-caseating granulomas[DAVIDSONS]
Over 90% of cases affect the lungs, but the condition can
involve almost any organ
Sarcoidosis is a multisystem inflammatory disease of
unknown etiology that predominantly affects the lungs and
intrathoracic lymph nodes.](https://image.slidesharecdn.com/sarcoidosis-190819081147/85/Sarcoidosis-5-320.jpg)
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Sarcoidosis
- 1. Sarcoidosis PROF.DR. MD.KHAIRUL HASSANJESSY PROFESSOR OF RESPIRATORY MEDICINE NIDCH
- 3. Introduction The diversityof the possible clinical manifestations is such that a practitioner in almost any branch of medicine may be called upon to make the diagnosis. All kinds of organ involvement are possible with the exception of the adrenals.
- 4. Introduction The serousmembranes are rarely involved with the exception of the pleura. The disease may present in acute, sub-acute or chronic form. Onset of disease occurs most commonly between 20 & 40 years of age with variable female predominance.
- 5. Defn Sarcoidosis isa multisystem granulomatous disorder of unknown aetiology that is characterised by the presence of non-caseating granulomas[DAVIDSONS] Over 90% of cases affect the lungs, but the condition can involve almost any organ Sarcoidosis is a multisystem inflammatory disease of unknown etiology that predominantly affects the lungs and intrathoracic lymph nodes.
- 6. Sarcoidosis is manifestedby the presence of noncaseating granulomas (NCGs) in affected organ tissues.
- 7. The modern historyof sarcoidosis In 1899, the pioneering Norwegian dermatologist Caesar Boeck describe skin nodules characterized by compact, sharply defined foci of "epithelioid cells with large pale nuclei and also a few giant cells . Thinking this resembled sarcoma, he called the condition "multiple benign sarcoid of the skin.
- 9. Epidemiology All racial. All ethnic groups. All ages (with the incidence peaking at 20 to 39 years). M-F ratio 2:1.
- 10. Epidemiology It ismore often described in colder parts of northern Europe More common and more severe in those from a West Indian or Asian background Eskimos, Arabs and Chinese are rarely affected The tendency for sarcoid to present in spring and summer
- 11. Etiology The causeremains elusive Have some role of infective agents, including mycobacteria, propionibacteria and viruses Genetic susceptibility is supported by familial clustering; a range of class II HLA alleles confer protection from or susceptibility to the condition Sarcoidosis occurs less frequently in smokers
- 12. The incidence Thehighest annual incidence in northern European countries 5 - 40 / 100,000. In Japan, the annual incidence 1 - 2 / 100,000. Among black Americans is roughly 3 times that among white Americans (35.5 / 100,000, as compared with 10.9 / 100,000.
- 13. Pathophysiology T CELLS PLAYA CENTRAL ROLE IN THE DEVELOPMENT OF SARCOIDOSIS, AS THEY LIKELY PROPAGATE AN EXCESSIVE CELLULAR IMMUNE REACTION.
- 14. The cause ofsarcoidosis is unknown. Efforts to identify a possible infectious etiology have been unsuccessful.
- 15. Genetic andenvironmental factors seem to play a role. As yet, no bacterial, fungal, or viral antigen has been consistently isolated from the sarcoidosis lesions. Sarcoidosis is neither a malignant nor an autoimmune disease.
- 16. The following havebeen suggested as possible candidates that might play a role in causing sarcoidosis: Mycobacteria, such as Mycobacterium tuberculosis, and atypical pathogens have been suggested. Fungi and viruses, particularly Mycoplasma, Chlamydia, and Epstein-Barr virus, have been unconvincingly implicated.
- 17. Environmental Causes Someof the earliest studies of sarcoidosis reported associations with exposures to irritants found in rural settings, such as emissions from wood-burning stoves and tree pollen. More recently, associations with sarcoidosis and exposure to inorganic particles ,insecticides ,and moldy environments have been reported. Occupational studies have shown positive associations with service in the U.S. Navy ,metalworking ,firefighting ,and the handling of building supplies.
- 18. Genetic Features Familialsarcoidosis was first reported in 1923 in two affected sisters . No formal twin study has been reported, but the concordance appears to be higher in monozygotic twins than in dizygotic twins . In A Case-Control Study, patients with sarcoidosis stated 5 times as often as control subjects that they had siblings or parents with sarcoidosis.
- 20. Presentation dependson the extent and severity of the organ involved. Approximately 5% of cases are asymptomatic and incidentally detected by CXR. Systemic symptoms occur in 45% of cases such as : Fever. anorexia Fatigue. Night sweats . Weight loss . Pulmonary symptoms: dyspnea on exertion, cough, chest pain, and hemoptysis (rare) occur in 50% of cases.
- 21. Presentation Possibe presentationin 3 ways Asymptomatic (Asymptomatic bilateral hilar lymphadenopathy) Acute (Erythema nodosum syndrome) Chronic (Cough & Dyspnoea)
- 22. Clinical features Insidious onsetis characterized by- Cough Exertional breathlessness Radiographic infiltrates Chest auscultation is often unremarkable
- 23. Clinical features Fibrosisoccurs in 20% of cases of pulmonary sarcoidosis May cause a silent loss of lung function Pleural disease is uncommon and finger clubbing not a feature
- 24. Asymptomatic bilateral hilar lymphadenopathy Common presentation Incidental finding on a chest X-ray
- 25. ERYTHEMA NODUSAM Erythema nodosumsyndrome Presents abruptly with fever, joint pains and tender red nodules on the shins Often associated with hot, swollen, exquisited tender ankle joints and other polyarthragia
- 26. Cough & Dyspnoea Most common symptom Requires treatment Onset usually gradual Often mistaken for a chest cold until a chest radiograph is obtained
- 27. Presentation of Sarcoidosis Asymptomatic – abnormal routine chest radiograph ( ~30%) or abnormal liver function tests Respiratory & constitutional symptoms ( 20 - 30%) Erythema nodosum & arthralgia ( 20 – 30%) Ocular symptoms ( 5 – 10%) Skin sarcoid (including lupus pernio) (5%) Superficial lymphadenopathy ( 5%) Others (1%) – e.g. hypercalcaemia,diabetes insipidus, cranial nerve palsies, cardiac arrhythmias, nephrocalcinosis
- 28.
- 29. Löfgren's syndrome An acutepresentation consisting of: • Erythema nodosum • Peripheral arthropathy • Uveitis • Bilateral hilar lymphadenopathy (BHL) • Lethargy • Occasionally fever occurs in 9 to 34% of patients (often seen in young women)
- 31. Heerford's syndrome : Anterior Uveitis Fever Parotid enlargment Facial palsy
- 35. Clinical features ofsarcoidosis Organ system involvement Symptoms or presentation Pulmonary Dyspnoea, cough, wheezing, haemoptysis Upper airway Dyspnoea, nasal congestion, polyps, hoarseness Dermatologic Nodules, papules, plaques, erythema nodosum, lupus pernio, kleoid scar Occular Photophobia, tearing ,pain, loss of vission, lacrimal gland enlargement Rheumatologic Polyarthropathy, monoarthropathy, myopathy
- 36. Clinical features ofsarcoidosis Organ system involvement Symptoms or presentation Neurologic Cranial nerve palsy, headache, hearing loss, paresthesia,seizures,meningitis,encephalopathy, Space-occupying lesion Cardiologic Syncope, dyspnoea, conduction disturbness, congestive heart failure, arrythmia, cardiac temponade Gastrointestial Dysphagia, abdominal pain, Jaundice, hepatomegaly, spleenomegaly
- 37. Clinical features ofsarcoidosis Organ system involvement Symptoms or presentation Haematologic Lymph node enlargement, hyperspleenism ( thrombocytopenia, leukopenia, anaemia) Renal Kidney failure, calculi Endocrine & metabolic Diabetes insipidus, hypercalcaemia
- 39. Although not life-threatening,but can be emotionally devastating. Erythema nodosum may occur. Lupus pernio is the most specific associated cutaneous lesion. Violaceous rash is often seen on the cheeks or nose. Osseous involvement may be present. Maculopapular plaques are possible. Cutaneous Involvement
- 40. LUPUS PERNIO Lupuspernio is more common in women than in men and is associated with chronic disease and extrapulmonary involvement.
- 41. ERYTHEMA NODUSAM Erythema nodosumoccurs in about 10% of patients with sarcoidosis and usually lasts for about 3 weeks. Biopsy specimens of erythema nodosum lesions show nonspecific septal panniculitis, which neither confirms nor negates the diagnosis of sarcoidosis.
- 42.
- 43. 10% ofall patients with sarcoidosis have elevated serum aminotransferase and alkaline phosphatase levels. A cholestatic syndrome characterized by pruritus and jaundice, hepatic failure, or portal hypertension can develop (liver involvement is usually clinically silent). Detection of hepatic and splenic lesions on CT is described in 5% and 15% of patients. 60% of patients with hepatic manifestations of sarcoidosis have constitutional symptoms such as fever, night sweats, anorexia, and weight loss. Portal hypertension with variceal bleeding, a hepatopulmonary syndrome with refractory hypoxemia, and cirrhosis leading to liver failure occur in only 1% of patients with sarcoidosis. Liver and Spleen Involvement
- 44. CNS isinvolved in up to 25% of patients with sarcoidosis who undergo autopsy, but only 10% of all patients with sarcoidosis present with neurologic symptoms. The most common problems: cranial-nerve palsies. Headache. Ataxia. cognitive dysfunction. Weakness. seizures. CSF Analysis : nonspecific lymphocytic inflammation. measuring ACE levels . oligoclonal immunoglobulin bands in the CSF are elevated, making it difficult to differentiate sarcoidosis from multiple sclerosis. Magnetic resonance imaging (MRI) Neurologic Involvement
- 45. The eyeand adnexa are involved in 25 -80% of patients with sarcoidosis,this necessitating routine slit-lamp and fundcopic examination. Anterior or posterior granulomatous uveitis . Conjunctival lesions and scleral plaques may also be noted. Ocular involvement may lead to blindness if untreated. Ophthalmologic Complications
- 46. Ophthalmologic Complications Anterior uveitis (isthe most common manifestation) chronic anterior uveitis, with insidious symptoms leading to glaucoma and vision loss, is more common than acute anterior uveitis.
- 47. Cardiac manifestations Heartfailure from cardiomyopathy rarely occurs. Heart block and sudden death may occur. Approximately 25% of patients may have NCGs at autopsy, but fewer than 5% have clinical cardiac disease. Okada et al reported on cardiac infiltration associated with a novel heterogenous mutation (G481D in CARD15) in early-onset sarcoidosis.
- 49. Initial Investigations forpulmonary sarcoidosis Routine haematological & biochemical tests Chest radiography.Obtain all previous chest radiograph Tuberculin Skin test Sputum studies for cytology and cultures for bacteria,fungi and mycobacteria
- 50. Initial Investigations forpulmonary sarcoidosis Pulmonary function tests,with spirometry (forced vital capacity, forced expiratory volume in one second), and carbon monoxide diffusing capacity. Review any previous studies Opthalmic examination with slit lamp is recommended Then patient specific studies as indicated
- 51. Investigations CBC- lymphopenia Liver function test- deranged S. Ca+2 - increased (reflecting increased formation of calcitrol – 1,25-dihydroxyvitamin D3 – by alveolar macrophages Serum ACE - can assist in monitoring the clinical course The presence of anergy (e.g. to tuberculin skin tests)
- 52.
- 53. Thoracic sarcoidosis Thehilar glands and the lungs are the organs most commonly affected in sarcoidosis By convention thoracic sarcoidosis is classified in four (04) stages on the basis of the appearance of the chest radiograph Stage I:Represents bilateral hilar lymphadenopathy Stage II: Bilateral adenopathy plus pulmonary opacities Stage III: Pulmonary opacities only Stage IV: Represents the development of irreversible pulmonary fibrosis
- 54.
- 55. Stage II isBHL and infiltrates
- 56. Stage III isinfiltrates alone
- 57. STAGE IV :ADVANCED FIBROSIS
- 59. Investigations In patientswith pulmonary infiltrates Pulmonary function testing- may show a restrictive defect accompanied by impaired gas exchange Exercise tests - may reveal oxygen desaturation Bronchoscopy - may demonstrate a ‘cobblestone’ appearance of the mucosa Bronchial and transbronchial biopsy - usually shows non-caseating granulomas The BAL fluid - typically contains an increased CD4:CD8 T-cell ratio HRCT of chest - reticulonodular opacities that follow a perilymphatic distribution, centred on bronchovascular bundles and the subpleural areas.
- 60. Initial Investigations forpulmonary sarcoidosis Routine haematological & biochemical tests Chest radiography.Obtain all previous chest radiograph Tuberculin Skin test Sputum studies for cytology and cultures for bacteria,fungi and mycobacteria
- 61. Initial Investigations forpulmonary sarcoidosis Pulmonary function tests,with spirometry ( forced vital capacity,forced expiratory volume in one second), and carbon monoxide diffusing capacity.Review any previous studies Opthalmic examination with slit lamp is recommended Then patient specific studies as indicated
- 62. For a confidentdiagnosis Erythema nodosum with BHL on chest X-ray is often sufficient , without recourse to a tissue biopsy Similarly A typical presentation with classical HRCT features may also be accepted
- 63. Usual radiographic manifestationof sarcoidosis Disseminated milliary lesions Disseminated nodular lesions Linear type of infiltration extending fan-wise from the hilum Diffuse & confluent patchy shadows Diffuse fibrosis
- 64. Usual radiographic manifestationof sarcoidosis Diffuse fibrosis with cavitation Changes similar to chronic tuberculosis as regards location and distribution Bilateral confluent massive opacities resembling areas of pneumonia Atelectasis
- 65. Unusual radiographic manifestationsof sarcoidosis Pleural lesions : Effusion Chylothorax Pneumothorax Pleural thickening Mediastinum : Adenopathy Isolated anterior, middle or posterior May be calcified and may show egg shell calcification Mediastinal emphysema
- 66. Unusual radiographic manifestationsof sarcoidosis Hilum: Unilateral hilar adenopathy ( 1-3%) and eggshell calcifications Lung: Lobar atelectasis, post obstructive bronchiectasis Cavitation Mycetaoma Vanishing lung Pulmonary nodule – single or multiple
- 67. Unusual radiographic manifestationsof sarcoidosis Cardiovascular lesions Pulmonar hypertension Superior vena cava obstruction Pulmonary artery obstruction by granuloma or enlarged lymph node Cardiomegaly Pericardial effusion Bony lesions including rib,sternum,thoracic vertebra
- 68. Tuberculin skin testing( MT) MT is a useful screening test Skin senstivity to tuberculin is depressed or absent in most patients A strongly positive reaction to one (01) TU virtually excludes sarcoidosis 2/3 rds of patients with active sarcoidosis fail to react to 100TU
- 69. Kveim test Ahelpful diagnostic procedure Particular saline suspensions of sarcoid tissue contain some component in varing amount which when injected intradermally in a patient with active sarcoidosis can provoke the slow development of an epithelioid cell ,granuloma of sarcoid type. Value of the test A positive test can be regarded as virtual proof of active sarcoidosis False positive fractions are rare, only 1-2% Positive tests are obtained in only 75% of patients with active disease
- 70. Kveim test Natureof the test substance Potent antigen prepared from human sarcoid tissue Usually from a cervical gland Rarely from spleen ( Spleenectomy done for spleenomegaly due to sarcoidosis Method of testing 0.1 ml – 0.2 ml intradermally Site The forearm is usually used For cosmetic reasons the upper and outer thigh may be used in females
- 71. KVEIM test Reading &Interpretation Within 4 weeks – a positive test will show a purplish red nodule at the site of injection Biopsy at 4-6 weeks reveals sarcoid tissue on histological examination Notes Corticosteroid therapy depresses the reaction If at all possible ,treatment should not be given until the test is read Problems: 4-6 weeks delay in diagnosis Difficulty in obtaining antigen Potential presence of HIV infection in the human tissue suspension Current avilability of more specific test
- 72. KVEIM TEST Inference: The Kveim reaction remains as immunological puzzle Response to kveim testing is uniform in all geographical areas It leads to support the concept of a single disease, rather than a syndrome and a single aetiology rather than a multiple one
- 73. Pulmonary function testing Reduction in lung volume Decrease pulmonary compliance Reduction in CO transfer factor Typical restrictive abnormalities in more advanced disease
- 74. Tissue biopsy Oftencrucial to diagnosis A search for superficial abnormalities (e.g. palpable lymph node,compatible skin lesion) should be looked for 100% positive in – epitrochlear lymph node enlarged, parotid glands,subcutaneous nodule,mediastinal lymph biopsy etc. 90% positive in – palpable scalene,inguinal,axillary lymph node biopsy 80% positive in – lung ( transbronchial),liver biopsy Lymph node & liver biopsies have a higher incidence of false positive diagnosis
- 75. SACE Not specific SACE in patients with active sarcoidosis reflects the granulomatous load of the body Elevation have been noted in various other disease including – milliary tuberculosis - atypical mycobacterial infection - Gaucher’s disease -leprosy -HIV infection -Hepatitis -Histoplasmosis
- 76. SACE Valuable inthe assessment of disease activity and response to treatment May be of value to diagnose in a difficult case Interest in research studies
- 77. 67Ga scanning Increaseduptake is found in the majority of patients with pulmonary tuberculosis A negative scan does denote inactivity of disease PROBLEMS: Costly Substantial amount of radiation exposure Not specific because any inflammation will show isotpoe uptake Not justified for routine examination
- 78. BAL Normally Balfluid contain mostly alveolar macrophage ( approximately 90%), fewer lymphocyte(10%) and small numbers of neutrophils ( 1-2%) In patients with sarcoidosis, there is an increase in the proportion of lymphocytes A high intensity alveolitis is defined as a lymphocyte differential count greater than 28% A low intensity alveolitis is one with a lymphocyte differential count less than 28% Intensity of alveolitis shows some forecast of the prognosis of disease
- 79. ABG analysis Tobe done, if there is dyspnoea
- 80. Serum calcium– increased 24 hours urinary calcium – increased ( Hypercalciuria occurs more frequently than hypercalcaemia)
- 81. Transbronchial biopsy Generallyused in pulmonary sarcoidosis Accepted method Relatively safe & easily obtainable False positive diagnosis are rare At least four biopsy should be taken Patients who do not have radiological evidence of parenchymal disease- positive in 50%- 60% of cases Who have radiological evidence of parenchymal disease – positive in 85 % to 90% of cases
- 82. Laboratory Studies Routinelab evaluation often is unrevealing. Hypercalcemia or hypercalciuria may occur (non caseating granulomas secrete 1,25 vitamin D). Hypercalcemia is seen in about 10-13% of patients, whereas hypercalciuria is 3 times more common. An elevated alkaline phosphatase level suggests hepatic involvement. Angiotensin converting enzyme (ACE) levels may be elevated.
- 83. NCGs secreteACE, which may function as a cytokine. Serum ACE levels are elevated in 60% of patients at the time of diagnosis. Levels may be increased in fluid from bronchoalveolar lavage or in CSF. Sensitivity and specificity as a diagnostic test is limited (60 and 70%, respectively). There is no clear prognostic value. Serum ACE levels may decline in response to therapy. Decisions on treatment should not be based on the ACE level alone.
- 84. Biopsy specimen Abiopsy specimen should be obtained from the involved organ that is most easily accessed, such as the skin, peripheral LN, lacrimal glands, or conjunctiva. If diagnosis requires pulmonary tissue, transbronchial biopsy by means of bronchoscopy has a diagnostic yield of at least 85% when multiple lung segments are sampled .
- 85. Sarcoidal granulomashave no unique histologic features to differentiate them from other granulomas. Special stains for acid-fast bacilli and fungi, as well as cultures of such organisms, are essential. If the results of lung biopsy with bronchoscopy are negative and other organs are not obviously involved, biopsy of intrathoracic lymph nodes, which are often enlarged in patients with sarcoidosis ,may be necessary to confirm the diagnosis.
- 86. The central histologicfinding is the presence of noncaseating granulomas with special stains negative for fungus and mycobacteria.
- 87. Assessing the patient’ssymptoms Is the disease interfering with the activity of daily living to such an extent that treatment is justified? So the degree of incapacity or abnormality objectively documented ( e.g. pulmonary function test, chest radiograph, abnormalities of liver function or serum calcium)? Do serial observations and measurements and the clinical picture suggest active and progressive disease? Have all resonable efforts been made to obtain previous chest radiographs and other clinical measurements ?
- 88. Activity of disease Important for prognosis and therapy Difficult to define Clinical & laboratory findings are the gold standard for activity, but there are very imprecise Basically three ( 03) tests used to assess the activity 1.Measurement of of percentage of lymphocytes in BAL 2. 67Ga scanning 3. Serum ACE concentration – these are not specific - important for research purpose In stage III & IV sarcoidosis assessment of disease progression is done by 1.measurement of lung volume 2.serial chest X-ray 3.CO transfer factor
- 93. Differential Diagnosis Hilar infiltrates: Tuberculosis. Lymphoma Eosinophilic granuloma Fungal infection Lung cancer
- 94. Differential Diagnosis NoncaseatingGranuloma on a biopsy : Berylliosis Catscratch disease Fungal infection Hypersensitivity pneumonitis Leprosy Primary biliary cirrhosis Tuberculosis.
- 95. Strategy for investigationof suspected sarcoidosis Diagnosis of pulmonary sarcoidosis depends on A compatible clinical picture Histological evidence of non-caseating granulomas in affected tissues or a positive kveim reaction The exclusion of other causes of granulomatous disease e.g. tuberculosis or beryllium disease
- 96. Sarcoidosis is thelikely diagnosis if Bilateral hilar lymphadenopathy and erythema nodosum occur in a tuberculin negative young woman Bilateral symmetrical hilar adenopathy in an otherwise asymptomatic person who may or may not have erythema nodosum or uveitis Reticulonodular changes are present on chest X-ray of an asymptomatic 20-40 year old patient with normal chest examination
- 97. Sarcoidosis is thelikely diagnosis if A diagnosis of sarcoidosis is reasonably certain without biopsy in patients who present with Löfgren's syndrome.
- 98. Strategy for investigationof suspected sarcoidosis At times the clinical presentation is sufficiently typical that the diagnosis can be made without biopsy
- 99. In case ofobscure pulmonary infiltrates If early therapeutic intervention seems to be necessary – transbronchial biopsy may establish the diagnosis If early therapeutic intervention seems not be necessary- kveim test may establish the diagnosis within 4-6 weeks In extrapulmonary sarcoidosis biopsy should be tried from most accessible site
- 101. Management Patients withacute illness and erythema nodosum are treated with NSAIDs If disease is severe, a short course of corticosteroids The majority of patients enjoy spontaneous remission If there is no evidence of organ damage, systemic corticosteroid therapy can be withheld for 6 months
- 102. Therapy Steroids arecurrently considered the most effective treatment for sacoidosis Steroids can suppress the manifestations of active sarcoidosis in nearly every case except sarcoidosisof the CNS Prednisolone is the most effective amonst steroid preparation It is not curative Natural course of disease seems to be uninfluenced by therapy
- 103. Indication of prednisolone Evidence of significant impairment of function of a vital organ ( heart, lung, brain, kidney or eye) Symptomatic stageIII pulmonary sarcoidosis Stage I & Stage II disease usually resolve spontaneously and treatment is seldom required Occasionally patients with persistent erythema nodosum, pyrexia and arthralgia or iridocyclitis require oral corticosteroid therapy for short period
- 104. Indication of prednisolone Persistenthypercalcaemia or hypercalciuria Uveitis Diffuse cutaneous lesion Parotid and lacrimal gland enlargement Symptomatic spleen involvement
- 105. Indication of prednisolone During or following a period of observation if any….. A) Symptoms develops B) There is significant radiographic or functional deterioration C) At the end of observation period there has been no significant improvement
- 106. Management Topical steroidsmay be useful in- Mild uveitis Inhaled corticosteroids have been used to - Shorten the duration of systemic corticosteroid use in asymptomatic parenchymal sarcoid Patients should be warned that strong sunlight might precipitate hypercalcaemia and endanger renal function
- 107. Management In patients withsevere disease- Methotrexate (10–20 mg/week) Azathioprine (50–150 mg/day) Specific tumour necrosis factor (TNF)-α inhibitors have been effective
- 108. Management In patient withcutaneous sarcoid with limited pulmonary involvement - Chloroquine Hydroxychloroquine Low-dose thalidomide may be useful Selected patients may be referred for consideration of single lung transplantation.
- 109. Cyclosporin –a logic choice Chloroquine , Hydroxychloroquine & Methotrexate – partly effective treatment Azathioprine – useful for pulmonary or eye sarcoidosis Cyclophosphamide – effective agent for sarcoidosis , but toxicity ( haemorrhgic cystitis & bladder cancer) prohibit its routine use except for neurosarcoidosis Tetracycline – monicycline and doxycycline may be useful for skin sarcoidosis Thalidomide and pentoxifylline ( against TNF) Monoclonal antibodies – Infliximab Whole brain radiation – used successfully for sarcoid meningitis
- 110. Management strategy ofpulmonary sarcoidosis Relatively asymptomatic patient Reiview history, physical examination and radiology at 2, 6, and 12 months, with pulmonary function tests if worse, or every year If the patient is worse treatment is indicated If the patient is not worse, repeat the tests annually for 2 years If the patient is worse after 2 years, treatment is indicated If the patient is not worse after 2 years, reassure him/her that the disease is in remission and unlikely to worsen Plan further follow up if patients status is equivocal
- 111. Symptomatic ,incapacitated patient Obtain histological support for the diagnosis Obtain baseline measurements of pulmonary function,current chest radiograph and other parameters of disease Begin prednisolone 40 mg/day for 2 weeks, then 30,25,20 mg/day each for 2 weeks to complete 2 months Document improvement in chest radiograph, pulmonary function and symptoms or other relevant clinical parameters. Objective data are important.If there is no improvement , rapidly taper prednisolone and stop giving it If the patient is improved, continue maintenance dose of 15mg/day for 8 months ( 10 mg/day may be sufficient)
- 112. Symptomatic ,incapacitated patient Document maximal improvement in chest radiographs and pulmonary function before further tapering and at the end of treatment Taper by 2.5 mg/month over 3-4 months to complete at least 1 year of treatment Observe for relapse after 2, 6 and 12 months. Objective data are required to indicate relapse Can begin retreatment with lower dose of 30 or 20 mg/day or even reinstitute previously adequate maintenance dose If several documented relapses occur, long term low dose treatment may be required for 10-20 years, or for life
- 113. Course and prognosis Usually sarcoidosis is a benign, self limited disease that resolves spontaneously Occasionally, the disease causes serious function limitation – most often pulmonary – may be life threatening Spontaneous remission upto 3 years – 30% to 50% Relatively stable – 20% to 30% Progression over next 5 to 10 years – 30 %
- 114. Course and prognosis Erythemanodosum syndrome is usually self limiting and has a good prognosis The older the age at onset, the greater the chance of chronicity
- 115. Prognosis The overall mortalityis low (1–5%) Bad prognostic factors- Age over 40 years Afro-Caribbean ethnicity Persistent symptoms for more than 6 months The involvement of more than three organs Lupus pernio A stage III/IV chest X-ray
- 116. Complications Bronchiectasis Aspergilloma Pneumothorax Pulmonary hypertension Cor pulmonale
- 118. COURTESY BY…… Dr. Md.Mofazzal Haider Siddique Resident, Phase –B MD (pulmonology) NIDCH Dr. MERAZUL ALAM MBBS, BCS DTCD STUDENT NIDCH Dr. Abu Hena Md. Masrur Resident, Phase –A MD (pulmonology,
- 119. Thank You I amStill Learning……