Sarcoidosis presented by Dr.Md.Saddam Hossain

Sarcoidosis
presented by
Dr.Md.Saddam Hossain
MBBS, BCS, CCD - diabetics( Birdem)
FCPS part 2 ( Medicine), PGT ( Gastro)

1. Sarcoidosis Presented by- Dr. Saddam Hossain Medical Officer (Department of Medicine) Sarkari Karmachari Hospital, Fulbaria , Dhaka.

2. Definition Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology , that is characterized by the presence of non- caseating granulomas.

3. Epidemiology More frequently occurs in colder parts of northern Europe. More common and more severe in those from an African Caribbean background. The tendency for sarcoidosis to present in the spring and summer. Women are more susceptible than men. 5.5% Familial susceptibility.

4. Pathophysiology Infective agents including- Mycobacteria Propionibacteria Virus Genetic

5. Clinical Features Lung- Lung involvement are seen in 90% of sarcoidosis patients. These include- Cough Exertional breathlessness Bilateral hiler lympadenopathy Lung fibrosis Pleural involvement (uncommon)

6. Clinical Features Skin- Skin involment are found in one third of sarcoidosis patients. The classic cutaneous lesions include- Erythema nodosum Maculo-papular lesions Hypo or hyperpigmentation Keloid formation Sub cutaneous nodules Lupus pernio (bridge of nose, area beneth eyes, checks)

7. Lupus pernio : Lupus pernio is a cutaneous manifestation of sarcoidosis. It presents as a violaceous lesion or shiny nodules over the head and neck but is predominantly localized to the nose, cheeks, and ears.

8. Erythma nodosum : Non- supporative , painful, palpable, erythematous, nodular lesion in the skin most commonly seen on shins.

9. Clinical Features Liver: Liver involvement is identified in about one half of sarcoidosis patients. These include- Hepatomegaly Intrahepatic cholestasis Portal hypertension Ascites Esophageal varices (PTO)

10. Clinical Features 6 . Elevated liver enzymes- ★Alkaline phosphatase mostly ★Slightly elevated transaminases ★Elevated bilirubin is a marker of advanced liver diasease

11. Clinical Features Bone marrow & Spleen Most common hematologic menifestation are- Lymphopenia Anemia (20%) 1/3rd sarcoidosis patient have granuloma in bone marrow examination Splenomegaly ( 5-10%) Splenic biopsy reveals Granuloma (60%)

12. Granuloma of Sarcoidosis Hallmark of sarcoidosis is non caseating granuloma, composed of a central core of epithelioid histocytes & multinucleated giant cells. Activated T cell & macrophages accumulate at the site of inflammation. Release of chemo attractants lead to cellular proliferation and granuloma formation. Progressives granulomatous inflammation leads to injury dysfunction & destruction of the affected organ.

13. Clinical Features Eyes : it includes Anterior uveitis Sicca syndrome

14. Sicca Syndrome : Immune mediated inflammation of salivary, lacrimal & sweat gland. C/F: Dry mouth Dry eye Dry vagina No arthritis (It is the differentiating point of Sjogren syndrome)

15. Clinical Features Lofgren syndrome : Acute presentation consisting of- Fever ( Occasional) Erythma nodosum Peripheral Arthralgia Bilateral hiler adenopathy (Occurs 9 to 34% of sarcoidosis patients)

16. Clinical Features Renal : < 5% involvement Nephrocalcinosis Hyper- uricemia Renal stone

17. Clinical Features Nervous system : 5-10% involvement of sarcoidosis patients Pachymeningitis Space occupying lesion Cranial nerve palsy Diabetes insipidus Optic neuritis Seizure Mononeuritis multiplex Peripheral neuropathy Cognitive impairment

18. Clinical Features Herefordt's syndrome : Facial palsy(LMN), accompanied by fever, uveitis & parotid gland enlargement.

19. Clinical Features Musculosceletal : Osteoporesis Arthopathy

20. Overview

21. Investigations CBC : Decreased Hb Lymphopenia Liver function test : Elevated S.bilirubin , ALP, SGPT S. Calcium : Elevated Serum angiotensin-converting enzyme (ACE) : Elevated (used in monitoring of clinical course of disease ) Xray chest : Bilateral hiler lymphadenopathy, pulmonary infiltrate, pulmonary fibrosis Lung Function Test : Restrictive defect

22. Investigations Bronchoscopy : May demonstrate a ‘ cobblestone’ appearance of the mucosa. Bronchial and transbronchial biopsies : Usually show non- caseating granuloma. Bronchoalveolar lavage : Fluid typically contains an increased CD4:CD8 T-cell ratio.

23. Investigations HRCT : Appearances include reticulonodular opacities that follow a perilymphatic distribution centred on bronchovascular bundles and the subpleural areas. FDG-PET scanning : can detect extrapulmonary disease.

24. Investigations Gallium Scan : Panda sign

25. Investigations Diagnosis should be confirmed by histological examination of the involved organ. The presence of anergy (e.g. to tuberculin skin tests) may support the diagnosis. The occurrence of erythema nodosum with BHL on chest X-ray is often suffcient for a confident diagnosis, without recourse to a tissue biopsy

26. Investigations Cytoplasmic inclusion : Asteroid bodies are star shaped cytoplasmic inclusions.

27. Investigations Schaumann bodies : Large concentric calcifications that form within the cytoplasm of giant cells

28. Investigations

29. Complications of Sarcoidosis Respiratory failure Renal failure Blindness Haemoptysis (secondary to mycetoma ) Heart block Cardiomyopathy Pulmonary hypertension Sudden death

30. Management of Sarcoidosis Acute illness and erythema nodosum : ★ NSAID ★If symptoms are severe- short course of glucocorticoids ( 2 weeks of prednisolone 5-15 mg daily)‌

31. Management of Sarcoidosis The majority of patients enjoy spontaneous remission and so, if there is no evidence of organ damage, systemic glucocorticoid therapy can be withheld for 6 months However, prednisolone (at a starting dose of 20–40 mg/day) should be commenced immediately in the presence of hypercalcaemia , pulmonary impairment, renal impairment and uveitis. Topical glucocorticoids may be useful in cases of mild uveitis, and inhaled glucocorticoids have been used to shorten the duration of systemic glucocorticoid use in asymptomatic parenchymal sarcoidosis. Patients should be warned that strong sunlight may precipitate hypercalcaemia and endanger renal function.

32. Management of Sarcoidosis In patients with severe disease, Methotrexate (10–20 mg/week), Azathioprine (50–150 mg/day) and specific tumour necrosis factor alpha (TNF- α) inhibitors have been effective. Chloroquine , Hydroxychloroquine and low-dose Thalidomide may be useful in cutaneous sarcoidosis with limited pulmonary involvement.

33. Management of Sarcoidosis Selected patients may be referred for consideration of single lung transplantation. If the disease is not improving spontenously 6 month after diagnosis, prednisone 30mg for 6 weeks, reducing to alternate day with 15mg for 6-12 month

34. Management of Sarcoidosis

35. Management of Sarcoidosis

36. Prognosis The overal mortality is low (1%–5%) and usualy reflects cardiac involvement or pulmonary fibrosis.

37. Thank you all…