Systemic Sarcoidosis Pulmonary Medicine Department Ain Shams University

At the end of this lecture the student should be able to: 1. Know the definition of systemic sarcoidosis. 2. Discuss the etiology and pathogenesis of systemic sarcoidosis. 3. Describe the clinical features. 4. Interpret the specific investigations. 5. List the differential diagnosis. 6. Discuss the treatment.

definition A multisystem granulomatous disorder of unknown origin ccc by activation of T lymphocytes and mononuclear phagocytes at sites of disease

Epidemiology More in women. More in blacks, esp. that of the skin. More between 20 – 45 years

Etiology UNKNOWN

Theories of aetiotogy Genetic: HLA, family clusters Mycobacterial Vitamin D metabolism abnnormalities.

PATHOLOGY Non caseating epithelioid granuloma typically at different stages of development. The core is formed dominantly of epithelioid cells with CD4 and mature macrophages interspersed The periphery CD4 &CD8 & macrophages. Giant cells containing cytoplasmic inclusion bodies are scattered. In the lungs they tend to form perivascular and peribronchial and resolve leaving no residue, hyalanized ghost or fibrosis.

Clinical picture > 2/3 asymptomatic. In symptomatic cases it most frequently affects the respiratory system (95%)with 20%eye and skin affection Therefore: –Pulmonary sarcoidosis –Extra pulmonary sarcoidosis

Pulmonary Sarcoidosis Classified according to clinical presentation into: –Acute sarcoidosis with erythema nodosum (fever+EN+bilat hilar LN+uveitis+ polyarth.) –Subacute sarcoidosis: S&S < 2 YEARS –Chronic sarcoidosis: S&S >2 years. –Fibrocystic sarcoidosis. –Dominant extrapulmonary sarcoidosis

Pulmonary sarcoidosis cont’d Respiratory symptoms take the form of dry cough, dyspnea, ill defined chest pain, wheezing if endobronchial disease In fibrocystic there is sputum,hemoptysis, P+++ and corpulmonale Rare pleural affection >in blacks

CXR Classifies pulm. Disease into: –Type 0: normal CXR –typeI: hilar LN –typeII: hilar LN +pulm infilterates. –Type III: pulm infiltrates –Type IV: extensive fibrocystic disease Rare X-ray pictures of PX, pl.eff.

Pulmonary function tests Normal Restriction Obstruction: endobronchial, laryngeal, fibrocystic decreased diffusion BHR to methacholine Decreased oxygen saturation and rarely carbon dioxide retention.

Extra-Pulm. Sarcoidosis Constitutional symptoms URT:severe nasal congestion, chronic sinusitis, laryngeal sarcoidosis. Occular: most commonly uveitis, also chorioretinitis, optic neuritis, conjunctivitis, keratoconj sicca Skin:erythema nodosum,lupus pernio,

Extra-Pulmonary sarcoidosis cont’d Alimentary tract: frequent liver affection with hepatitis plus severe pruritis. Rare GIT affection. Cardiac : arrhythmia, myopathy, rarely SVC obstruction. CNS:Facial & cranial neuropathy, aseptic meningitis, mass ….,DI, hypoth or pit. Involv, polyneuropathy, MNM.

Extra-Pulmonary sarcoidosis cont’d Hemopoietic system: LN, Splenomegaly, BM. Salivary and lacrimal glands:tender and enlarged Bone and joint: usually polyarthritis, punched out bony lesions with cystic changes

Extra-Pulmonary sarcoidosis cont’d Muscles: myopathy, rarely symptomatic Endocrine: hypercalcaemia and hypercalcuria( inc. activation of vit D), hypopit., DI., hypothalamic, epididimitis. Renal: calculi, nephrotic, CGN

Clinical assessment Tissue biopsy Kveim siltzback test Specific for every system: …. Cutaneous anergy ESR. CXR PFT. Serum ACE, Gallium scan, BAL CD4:CD8

TREATMENT SYMPTOMATIC AND LOCAL WHENEVER POSSIBLE If symptomatic or progressive pulmonary, threatened organ failure,.. Ttt in pulmonary and systemic dis. Is corticosteroids starting with 40 mg decreased in 5 mg increments every two weeks till 15 mg which are maintained for 6 – 8 month then tapered gradually by 2.5 mg every 2-4 wks while assessing for activity or relapse after 2, 6, 12 months

TREATMENT cont,d Chloroquine for mucocutaneous sarcoidosis in 6 months course to be rptd when needed at 6 months intervals Methotrexate in severe or refractory disease Heart lung transplantation Antifailure measures

Thank you You may now ask your questions