Pulmo Rhemu Collaboration template only.pptx

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1. Multidisciplinary Team Initiative CTD related ILD treatment RA-ILD Treatment Scleroderma Lung disease Biologic Workup Rheumatic Lung disease Registry formation

2. Connective Tissue Disease Estimated prevalence of ILD ILD pattern Frequency of CTD manifesting as Occult Myositis, Anti-synthetase syndrome 40% NSIP with OP , NSIP , UIP Often Rheumatoid arthritis 10% clinical, 30% subclinical UIP , NSIP, OP Less often Sjögren’s syndrome 40% NSIP, UIP, LIP Less often Systemic sclerosis 30–40% clinical, 80% subclinical NSIP, UIP Less often Systemic lupus erythematosus 8–12% DAH, NSIP ILD is infrequent Interstitial pneumonia with autoimmune features 100% NSIP, OP, NSIP with OP, UIP, LIP Always CTD related ILD Prevalence ( Arthritis Rheum  2010; 62 :1583–91.)

3. Why is ILD Screening for CTD improtant ? Of 114 patients referred to an ILD clinic, 15% of patients had a CTD as a consequence of evaluation for ILD ( Respir Med  2009; 103 :52–8.). Positive serologies (RAF, Low Titre ANA) have been reported in more than one-third of patients with non-CTD ILDs ( Respir Res  2017;18:161.) and should not be regarded as diagnostic for CTD-ILD . In Belgium, A multidisciplinary evaluation led to a change in the preliminary dx from IPF to CTD-ILD in 42% of cases ( Chest  2018). Similarly, at a US tertiary care clinic, multidisciplinary evaluation led to a change in diagnosis in 54% of patients referred with idiopathic pulmonary fibrosis (IPF) or a CTD-ILD ( Rheumatology ; 50 :489–93).

4. Scleroderma Lung management Cyclohpsphamide (oral, IV): Scleroderma Lung Study 1. Mycophenolate Mofetil (MMF): Slceroderma Lung Study 2. Azathiorpine (For maintenance only- MMF preffered). MMF Drug of Choice for Scleroderma ILD (Benificial in Skin as well) Steroids kept as low as possible , Start low if + anti Scl 70, Diffuse disease . Plz Start Using IV Cyclophosphamide for Lung ( Non expensive) Nintedinib

5. Scleroderma Lung (Second line) Rituximab 60 patients with diffuse cutaneous SSc-ILD, Rituximab vs Cyclophosphamide. Significant improvement in mean FVC% in the rituximab grp at 6 m (p 0.02). Both Skin and ILD improved in Rituximab group (P <0.05). A case-control study of 63 patients treated with rituximab and 25 matched controls found that rituximab significantly slowed FVC decline. Lepri et al conducted a retrospective assessment of rituximab efficacy in the treatment of CTD-ILD, including 23 patients with SSc. The PFT decline seemed to stabilize by the second year of follow-up. (Ann Rheum Dis. 2015;74(6):1188-1194. 55). (Clin Exp Rheumatol . 2016;34(5):181-185).

6. Tocilizumab Tocilizumab has been investigated as a treatment for SSc in two randomized trials, FaSScinate and FocuSSced trials. Patients in these trials had diffuse cutaneous SSc, with modified Rodnan skin score (m-RSS) of 15 to 40, the presence of tendon friction rubs, and elevated acute-phase reactant levels. Both Trials primarily for Skin: Failed to Improve Both trials failed to meet the primary endpoint (change from baseline in modified Rodnan skin score) but suggested that: Tocilizumab may have a benefit on lung function Tocilizumab potentially attenuated FVC decline (significantly fewer patients experienced a drop in FVC% compared with placebo at 48 weeks (P¼.0373).

7. Rheumatoid Arthritis Related ILD RA-ILD treatment is mainly initiated in clinically significant disease when there is evidence of disease progression. Corticosteroids daily oral dose, and tapered over months depending on the tolerability and clinical response, Methotrexate , Mycophenolate Mofetil (MMF), azathioprine, and cyclophosphamide as maintenance therapy. Cyclophosphamide in combination with methylprednisolone has shown some benefit in rapidly progressive, severe ILD and in RA-ILD with extensive UIP Rituximab, a B-cell-depleting anti-CD20 antibody, was used in 700 patients with RA; 56 had RA-ILD prior to rituximab treatment. A total of 68% of these patients improved or had stable pulmonary function after rituximab. Rituximab may be useful in patients with CTD-ILD with severe ILD refractory to other forms of immunosuppression. RA-ILD with a UIP pattern may have effect to antifibrotic agents. Nintedinib beneficial on slowing the decline of lung function in RA-ILD .

8. Interstitial Pneumonia with Autoimmune Features This term interstitial pneumonia with autoimmune features (IPAF) replaces idiopathic interstitial pneumonia (coined in 2015). Interstitial pneumonia by HRCT in a patient who does not meet the criteria for a connective tissue disease; but have features of CTD wether on clinical grounds or Serology. The clinical features include “mechanic hands”), distal digital tip ulceration, arthritis, morning joint stiffness ≥60 min, Raynaud's phenomenon, Gottron’s sign. The serologic domain includes circulating autoantibodies known to be associated with CTDs. Low-titre ANA, and low-titre rheumatoid factor are not included.

9. Biologic Agents Workup High Risk of re activating Latent TB (2.5% -15%) Increased Infections, Including PCP Anti TNF, Rituximab, Tocilizumab, Secukinumab (Lowest) Hepatitis B and C reactivation Latent TB Screening Chest Xray IGRA, Quantiferon, TB Spot, PPD If very suspicious of active TB, Sputum smear

10. L a tent TB (if positive, Treat) If positive, Treat with Latent TB Guid e lines Already Hepatotxic drugs used, (Two months of multiple heptotoxics) F ortnightly tests if ATT started, (LFTs, CBC, Creatinine) No Current Guidline for high dose, cs-DMARDs and recativation (Can be checked prospectively) Atleast 4 patientts with persistant increased Lung infections Pneumovax, Fluvax for CTD without Biologics 30 mg Delta and Septran – ANCA, Takayasu, Giant cell, Lupus ANCA associated vasculitis - Septran

11. Clinial Data registry An interactive database that collects, organizes, and displays healthcare information.  Aim : To evaluate and improve outcomes for a population defined by a particular condition, disease, or exposure. Registries, Specifically use observational study methods to collect and harmonize data about the treatment, outcomes, and well-being of patients who receive care over time. Registries aggregate large data sets and analyse trends or patterns in treatments and outcomes .

12. Rheumatic Lung Registry RA related ILD Sjogren related ILD Scelorderma ILD MCTD related ILD – Anti syntheatse Syndrome, Myositis related ILD