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Scleroderma Associated Pulmonary HTN

Scleroderma Associated Pulmonary HTN. August 13 th /03 Wael Batobara. History. 69 y female Ex smoker 8y {30 pack y} NIDDM Microalbuminuria & PVD HTN ,Dyslipidemia Admitted under Vascular Sx 15 th July/03 Rt Femoral – Popliteal Bypass June/03 Worsening Ischemic toe

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Scleroderma Associated Pulmonary HTN

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  1. Scleroderma Associated Pulmonary HTN August 13th /03 Wael Batobara

  2. History • 69 y female Ex smoker 8y {30 pack y} NIDDM Microalbuminuria & PVD HTN ,Dyslipidemia • Admitted under Vascular Sx 15th July/03 Rt Femoral – Popliteal Bypass June/03 Worsening Ischemic toe planed amputation

  3. History • Chest Medicine consult : preoperative evaluation • Exertional SOB & LL swelling 1/12 • SR: -ve orthopnea ,PND, Chest Pain ,Cough ,sputum , Wheeze ,Hemoptysis , Fever ,Wt loss • PMH: -ve IHD normal MIBI June/03 -ve Valvular disease -ve VTE ,Recurrent Pneumonias

  4. History • Medications At Home Glyburide , Metformin Lisinopril ,Nifidipine ,Pravastatin In Hospital Lasix , ASA • No H/O Travel ,Pets ,Occupational exposure • FH : unremarkable

  5. Examinations • No respiratory distress , Cyanosis • RR 16 Sat 90-92% at rest 74% walking • BP 130/80 HR 75 Afebrile • JVP 7 cm ASA with normal carotids

  6. Examinations • Lt parasternal lift apex 6th ICS MCL • S1+S2+S3+S4 Loud S2 • 3/6 PSM Lt sternal border increased with inspiration • Chest : -ve clubbing Good B/S Clear

  7. Examinations • Abdomen Hepatosplenomegaly ? Ascites • LL : Bilateral pitting edema -ve DVT signs • No Connective tissue diseases signs

  8. Investigations • CBC N • BUN ,Creatinine & Electrolytes N • U/A N • LFT , CK & TnT N • EKG Lt Axis ,Poor R wave progression

  9. Investigations • CXR & PFT • ABG PH 7.48 PCO2 43 Sat 86% PO2 49 HCO3 31 On room air • 2DE: LVH EF N Mild Diastolic Dysfunction pulmonary HTN RV SP 75 Moderate TR Dilated RV & RA

  10. Working Dx • Pulmonary HTN  Rt HF • Etiology ? Secondary Vs Primary • What Possible secondary causes?

  11. Something Unexpected • Anti-centromere Abs +ve 1 : 5120 • Other ENA –VE • 2nd Rheumatologic exam {By Rheumatology Team }  -ve • Rx  O2 , Diuretics • Referral to Pulmonary HTN Clinic

  12. Few Question That Needs Answers • How sensitive is Anticetromere Ab ? • Is any clinical predictors of worse outcome? Patient Characteristics Physical exam Laboratory tests

  13. Sensitivity & Specificity Of ACA • Retrospective Study • 397 Clinical Dx Systemic Sclerosis • 26% +ve Anti Scl70 Vs 22% +ve ACA Clinical Correlations & Prognosis Based on serum Abs in SS Arthritis-Rheum Feb 1988

  14. Sensitivity & Specificity Of ACA • 96% of +ve ACA were in Limited Scler. BUT only 43% of Limited Scler. Had +ve ACA • ACA +ve more Calcinosis & Telangectasias • Equal Pulmonary HTN

  15. Sensitivity & Specificity Of Anti Scl70 • 66% of +ve Anti Scl70 were Diffuse Scler. BUT Only 33% of Diffuse Scler. Had +ve Anti Scl70 • Anti Scl70  more Raynauds & IPF • Equal renal & cardiac events

  16. Patient Characteristics • Retrospective study • 189 female patients with normal initial CXR & 2DE • 63/189 developed Pulmonary HTN Annals Of NY Academy Of Science June 2002

  17. Patient Characteristics • What Clinical & Biological markers was predictive? Postmenopausal Status RR 5.2 CREST RR 2.8 HLA B35 RR 2.8

  18. Physical Examination • Prospective Study • Quantitative Nail fold Capillaroscopy in 10 Controls Vs 20 Limited SS • 18/20 +ve ACA & 8/20 Pulmonary HTN • Pulmonary HTN has significant decrease in capillary density > 2 groups ( p < 0.01) Asian Pacific Journal Of Allergy & Immunology June 1998

  19. Laboratory Investigations • Soluble Thrombomodulin Levels • In 34 Scleroderma with Pulm.HTN & 38 Scleroderma without Pulm.HTN & 2o Controls • Significant Elevations In SS with Pulm HTN • No Difference between SS without HTN & Controls • NO further elevation with worsening HTN Annals Of Rheum Dis Feb 2000

  20. Laboratory Investigations • Prospective Study • Survival in Patients with Pulmonary HTN • 40 Patients 24/40 Cyanotic heart disease 11/40 Primary Pulm.HTN 3/40 Schitosomiasis 2/40 CTD • Plasma vWF levels Chest November 1998

  21. Laboratory Investigations • 7/11 Primary HTN died over 1 year • 4/29 Secondary died over 1 year • Plasma vWF > 240% was 54% sensitive & 93% specific for predicting who is unlikely to survive

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