Sarcoidosis is a multisystem disorder characterized by the presence of noncaseatinggranulomas.
Most affected organ is the lung Skin, eyes and lymph nodes are frequently involved Acute or sub acute and self limiting Waxing and waning over years
Incidence and Prevalence
Disease can affect people of any age, race and gender.
20 to 40 years of age.
Most cases are Sporadic . 5 % of patients – FH of Sarcoidosis.
Pathophysiology Granulomas are Pathologic hall mark of sarcoidosis. Granuloma is an organized collection of macrophages.
granulomas form in response to antigens that are resistant to "first-responder" inflammatory cells such as neutrophils and eosinophils. The antigen causing the formation of a granuloma is most often an infectious pathogen or environmental agent, but often the offending antigen is unknown
The antigenic triggering agents cause activation of the Helper T-cell and Macrophages
Activated Helper T-cell and Macrophages produce IL-2,IFN and TNF complex interaction of cytokines Inflammatory response leading to granuloma
Giant cells in the central part of the granuloma.
Giant cells are fused Macrophages-Langerhans giant cells.
The central epithelioid and giant cells are surrounded by a rim of lymphocytes, mostly T-helper cells
All granulomas, regardless of cause, may contain additional cells and matrix. These include lymphocytes, neutrophils, eosinophils, fibroblasts and collagen [fibrosis].
CYTOPLASMIC INCLUSION BODY Schaumann body (arrow) is common in sarcoidosis but is nonspecific.
Clinical Manifestations 50% patients are asymptomatic Abnormal "routine" chest radiograph Symptomatic patients, with wide variety of symptoms Onset is usually insidious but can be acute
Clinical Manifestations Respiratory symptoms are most common Cough, chest discomfort, and dyspnea Symptoms reflect the specific organs involved by the granulomas
Lungs First site involved Begins with alveolitis involving small bronchi and small blood vessels Alveolitis either clears up spontaneously or leads to granuloma Fibrosis
Noncaseating granuloma in lung is the characteristic lesion of sarcoidosis.
CASEOUS NECROSISCellular destruction in TB granuloma appears as clumped debris (arrows). This necrosis does not occur in sarcoidosis.
M. tuberculosis BACILLI Caseous necrosis is most common in TB, but Gram negative, acid fast bacilli must be identified to make the diagnosis.
SUBPLEURAL GRANULOMA IN LUNG
Eyes 25% have eye lesions Blurred vision, pain, photophobia and dry eyes Chronic uveitis leads to glaucoma, cataracts and blindness Keratoconjunctivitis sicca Papilledema
PAPILLEDEMA Often associated with 7th nerve facial palsy.
Skin 33% have skin lesions LOFGREN'S SYNDROME; acute triad of erythemanodosum, joint pains, and bilateral hilaradenopathy
NAKED GRANULOMA Young granulomas (arrows) in the skin with no surrounding rim of mononuclear cells.
ERYTHEMA NODOSUM These reddish raised lesions.
Skin Lupus pernio- indurated blue purple swollen shiny lesions on nose, cheeks, lips, ears and fingers. Papules, nodules, and plaques Psoriatic like lesions
RAISED PLAQUESThese raised plaques are the result of coalescence of nodules.
PSORIASIS LIKE LESIONS These small white lesions closely resemble psoriasis.
Liver 33% have hepatomegaly or biochemical evidence of disease Symptoms usually absent Cholestasis, fibrosis, cirrhosis, portal hypertension, and the Budd-Chiari syndrome have been seen
Musculoskeletal Acute polyarthritis with fever is common Arthritis is self limited Chronic destructive bone disease with deformity is rare Muscle disease is rare
PUNCHED OUT LYTIC LESIONS Focal osteolytic lesions in the fingers are most common abnormality.
LACY TRABECULAR PATTERN Osteolysis has left a lacy trabecular pattern in this phalanx (arrow)
DEFORMING LESIONS Advanced sarcoidosis with osteolytic lesions of the distal forearm, wrist, and bones of the hand
SCLEROTIC LESION Rare and often in the axial skeleton.
NASAL BONE LESION Nasal sarcoidosis can lead to osteolysis of the nasal bone (arrows).
Heart 25% have cardiac involvement. Sarcoidosis can affect any part of the heart. Mostly Asymtomatic. Screening should be done to rule out cardiac involvement in Sarcoidosis.
Cardiac Sarcoidosis Arrhythmias Heart blocks- common Heart failure- Restrictive to Dilated CMP. Pericarditis- Effusions. MI SCD
Cardiac Sarcoidosis Difficult to Dx. Screening and Dx can be done by : ECG ,Holter monitoring ,ECHO Nuclear imaging (with thallium and/or technetium sestamibi) Cardiac PET/MRI Heart biopsy, rarely /Never-can miss Dx –patchy involvement of myocardium.
CNS Neurosarcoidosis affects 15 percent of sarcoidosis patients. Some people with neurosarcoidosis will recover completely. In others, sarcoidosis and related nervous system symptoms are chronic, lasting a long time or even a lifetime
nerve inflammation and damage peripheral neuropathy
Granulomas in the meninges (or more rarely in the brain) can lead to meningitis, hydrocephalus and neuroendocrine disorders .
Cranial nerves, and peripheral nerves can be involved 7th nerve facial palsy is most common Acute, transient, and can be unilateral or bilateral HEREFORDT'S SYNDROME; facial palsy accompanied by fever, uveitis, and enlargement of the parotid gland
MR IMAGE Temporal lobe sarcoid lesion (arrow)
Nervous System Optic nerve dysfunction-Blurring,doublevision,blindness. seizures Paresthesias Encephalopathy
CNS symptoms are not usually the first or only sign of sarcoidosis.
Rarely Neurosarcoidosis is the only sign of sarcoidosis. Isolated Neurosarcoidosis – Difficult to diagnose CT/MRI/PET SCAN/LP will aid in Dx. Biopsy rarely done.
Kidney Granulomatous interstitial nephritis produces renal failure Develops over a period of weeks to months Rapid response to steroid therapy Kidney stones (nephrolithiasis) and nephrocalcinosis are very unusual secondary to hypercalcemia and hypercalciuria
Kidney Increased calcium absorption in the gut Related to high levels of circulating 1,25-dihydroxy vitamin D produced by mononuclear phagocytes in granulomas
Lymph Nodes Lymphadenopathy Intrathoracic nodes enlarged in 75-90% patients including hilar nodes and paratracheal nodes. Peripheral lymphadenopathy
Enlarged B/L Hilar, RT paratracheal .
CALCIFIED LYMPH NODES late manifestation in 5% of patients.
PARACARDIAC LYMPH NODE
ABDOMINAL LYMPHADENOPATHY Multiple enlarged paraaortic, paracaval, and porta hepatis lymph nodes (arrows).
GASTRIC SARCOID Granuloma involves the gastric antrum leading to irregular nonspecific narrowing.
COLONIC SARCOID Irregular narrowing of the rectosigmoid has the appearance of inflammatory disease or malignancy.
ACE levels tested regularly to check the severity of the disease and to monitor the response to therapy.
Radiography CXR 3 classic patterns are seen. Type 1- bilateral hilaradenopathy with no parenchymal abnormalities. Type 2- bilateral hilaradenopathy with diffused parenchymal changes. Type 3- diffused parenchymal changes without hilaradenopathy.
STAGE I Thoracic lymphadenopathy. Normal lung parenchyma. (50%)
STAGE II Hilar and mediastinal lymphadenopathy. Abnormal lung parenchyma. ( 30% )
STAGE III Abnormal lung parenchyma. No lymphadenopathy. ( 15% )
STAGE IV Permanent lung fibrosis. (20%)
MILIARY SARCOIDOSIS CT shows well defined lung nodules less than 5mm in diameter. This pattern is rare.
ALVEOLAR SARCOIDOSIS Multiple lung masses are an unusual form of sarcoidosis, resembles lung metastases.
Lung Function Test Lung function abnormalities for interstitial lung disease with decreased lung volumes and diffusing capacities
Lymph nodes with rim (eggshell) calcification (arrow) are rare in sarcoidosis but common in silicosis.
MOST COMMON PATTERN Bilateral symmetric hilar and right paratracheal mediastinal adenopathy.
Diagnosis Difficult to differentiate from chronic infections, fungal diseases, T.B. and lymphoma. Based on combined clinical, radiologic and histologic findings. Laboratory tests seldom important
Diagnosis Identify noncaseatinggranulomas Transbronchial biopsies positive in 65-95%, even if no lung parenchymal abnormalities imaged. Tissue from mediastinoscopy positive in 95% Scalene node biopsy positive in 80%
Diagnosis KVEIM TEST-Like Mantoux. Involves injecting standardized preparation of sarcoid tissue material into the skin. Unique lump formed at the point of injection is considered positive for sarcoidosis.
Diagnosis Test not always positive Test material not approved for sale by FDA.
Differential Diagnosis 1.Hodgkin's disease: Mediastinal lymphadenopathy predominates in the anterior mediastinum and the paratracheal regions. When lymphadenopathy involves the hilar regions, it is usually asymmetric.
2. Pulmonary tuberculosis:
Nodal enlargement is unilateral in about 80% of tuberculosis cases.
Lymph nodes are less well demarcated than in sarcoidosis.
Lymphadenopathy is almost always associated with ipsilateral lung disease
Lymphadenopathy may be unilateral or bilateral and is usually associated with parenchymal disease. Hilar and/or paratracheal lymph nodes may be involved.
4.Bronchogenic carcinoma: Unilateral hilar lymphadenopathy is common. The appearance of the primary lung cancer (not visible in all cases) is often a large mass, which is unusual in sarcoidosis.
5.Metastatic lymph node enlargement: Primary cancer in the kidney, prostate gland, or the UGI tract usually involves the middle mediastinal lymph nodes.
Prognosis Good In 15-20% remains active or recurs intermittently. 50% have some permanent organ dysfunction
Treatment No known cure Corticosteroids, primary treatment for inflammation and granuloma formation. Prednisolone, 1 mg/kg for 4-6 weeks followed by slow taper over 2-3 months. Abnormal cardiac/Neuro/Ocular /Hypercalcemia/Multi system – Steriods must.
Treatment CutaneousSarcoidosis- Topical steriods/Hydroxychloroquine. Hydroxy chloroquine-200-400mg/day-Eye exam -6 monthly. MTX- Preni not tolerated/not effective/steriod sparing agent. MTX –Start with 10mg/week and maintain with 2.5 to 15mg/week.CBC,KFT,LFT/2months.
Infliximab-Monoclonal Ab to TNF- Improved lung function when given along with predni and MTX- recent RCT.
Initial PPD –Reactivation of TB.
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