intersticial pulmonary fibrosis and sarcoidosis

this topic includes all about these 2 diseases

1. INTERSTITIAL PULMONARY FIBROSIS By Shumaila Zahid ( Roll No. 32 )

2. IPF is a fatal lung disease characterized by an unpredictable decline of lung function due to lung fibrosis Progressive decline in lung function restricts routine physical activity Notable inter- and intra-patient variability in deterioration Prognosis is extremely poor- Median survival 2-5 years The cause of IPF is not known

3. Diagnosis 1. Laboratory investigations CBC (lymphocytosis) Serum Ca+2 levels LDH levels Serum ACE levels Anti CCP antibodies Chest X rays Show sub pleural reticual shadowing HRCT Patchy , Peripheral , subpleural and basal reticular patterns Honeycombing cysts Diagnostic test

4. Diagnosis PFTs FEV1/FVC ratio normal or Increased Lung volumes reduced Dynamic tests To document exercise intolerance , exertional arterial hypoxemia and hypocapnia Bronchoscopy In case of infection or malignant process 7. Lung biopsy . When diagnostic uncertainty or atypical features

5. Xray

6. HRCt

7. Management Pharmacological therapies Pirfenidone (slows the rate of decline of lung function and improves mortality ) ⁠ Ninetidanib Non pharmalogical therapy LTOT Lung transplant Mechanical ventilation Pulmonary Rehabilitation

8. Management Treatment of complications and comorbidities Acute exacerbation Corticosteroids GERD Advance disease central cynosis ⁠features of heart failure CA of lung

9. Pulmonary Sarcoidosis By Tayyba Qayyum ( Roll No. 33 ) Shumaila Zahid (Roll No. 32)

10. Sarcoidosis Chronic Multi system granulomatous disorder characterized by the presence pf non caseating granuloma Unknown cause 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

11. Risk factors Autoimmune disorder Infectious agents Mycobacteria Propionibacteria       Viruses

12. Clinical features more common in young women Cough Exertional Breathlessness Chest auscultation is unremarkable

13. Nasal cutaneous sarcoid lesion

14. Investigations • CBC- lymphopenia Liver function test deranged S. Ca+2 - increased (reflecting increased formation of calcitriol- 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) Chest Xray used to stage sarcoidosis

15. Investigations Pulmonary function testing show restrictive defect along with impaired gas exchange Exercise test may reveal O2 desaturation Bronchoscopy demonstrate cobblestone appearance of mucosa  Bronchial and Transbronchial biopsies show non caseating granuloma Bronchoalveolar lavage fluid contains increased CD4:CD8 T cell ratio. Characteristic HRCt include reticulonodular opacities

16. Treatment Pt with acute illness and erythema nodosum Nsaids and on occasion short course of Glucocorticoid .Topical glucocorticoid in case of mild uveitis and to shorten the duration of systemic GC use in asymptomatic parenchymal sarcoidosis. f no evidence of organ damage systemic glucocorticoid therapy can be withheld for 6 months. prednisolone(20-40mg/day) should start immediately in presence of hypercalcemia ,pulmonary impairment ,renal impairment and uveitis .Pt should be warned that strong sunlight may precipitate hypercalcemia and endanger renal function.

17. Treatment Pts with severe disease methotrexate(10-20mg/week),azathioprine(50-150mg/day and TNF-Alpha are effective. Chloroquine, hydroxychloroquine and low dose thalidomide useful in cutaneous sarcoidosis with limited pulmonary involvement. Selected patients may referred for lung transplant Overall mortality is low and usually reflects cardiac involvement or pulmonary fibrosis.