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Scleroderma Presenting with Pulmonary Hypertension

Scleroderma Presenting with Pulmonary Hypertension. Cara Fininzio, D.O. Research Symposium June 9, 2010. Background. Scleroderma (systemic sclerosis) is rare multisystem disease, causing fibrosis of multiple organ systems

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Scleroderma Presenting with Pulmonary Hypertension

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  1. Scleroderma Presenting with Pulmonary Hypertension Cara Fininzio, D.O. Research Symposium June 9, 2010

  2. Background • Scleroderma (systemic sclerosis) is rare multisystem disease, causing fibrosis of multiple organ systems • Skin, kidneys, lungs, GI tract, heart, and blood vessels can be affected • Two forms are identified but there can be some overlap

  3. Background • Diffuse cutaneous • Rapid development of symmetric skin thickening • Greater risk for other visceral disease early in course • Limited cutaneous • Symmetric thickening of skin on distal extremities/ face • Can have features of CREST

  4. Background • Diffuse: • Distal and proximal extremities, trunk, and face skin involvement • Raynaud’s phenomenon at onset or within 1 year of skin changes • Pulmonary involvement (fibrosis), renal, GI, cardiac • Antitopoisomerase I AB

  5. Background • Limited: • Distal extremity/ face skin involvement • Raynaud’s may precede by years • GI, PAH after 10-15 years of disease, biliary cirrhosis • Anticentromere AB

  6. Case Report

  7. History • A 69 year old Caucasian Female presented with worsening SOB and DOE for the past couple weeks • ROS: + orthopnea, SOB, DOE

  8. Past History • PMH- CHF/ Cardiomyopathy, HTN, Osteoporosis, Raynaud’s phenomenon, Pulmonary HTN, GERD, SAR, Lower Extremity Edema, Pruritis, Pneumonia September 2008 • PSH - Tonsillectomy as child • Allergies – None, NKDA

  9. Past History Medications: Lisinopril 5mg po daily Furosemide 20mg po daily Carvedilol 3.125mg po daily KCl 10meq po daily Omeprazole 20mg po daily Loratadine 10mg po daily Fosamax 70mg po q week Calcium + Vitamin D po daily FeSo4 325mg po BID

  10. Past History • Social- Married, retired, G4P3013, ~5 pack year tobacco history (quit 38 years ago), recovered alcoholic since 9/2008 (admits to a couple beers up to 2 pints of whiskey/ day in past) • Preventive – Colonoscopy 2007, Pap 2005, Mammogram 1/2009 all negative, Pneumonia Vaccine 2008, Influenza Vaccine, Measles and Varicella diseases ad child

  11. Past History • FxHx – Mother deceased in 80s (Breast CA at age 35), Father deceased age 67 with DM

  12. Previous Testing • Chest CT – Mild mediastinal lymphadenopathy 5-15mm nonpathologic, no hilar lympadenopathy, mild cardiomegaly • 2D echo – LVEF 30% (1/2009), pulmonary artery pressure of 40mmHg

  13. Previous Testing • PFT – FVC 2.87L 116% predicted, FEV1 2.08L 105% predicted, FEV1/FVC 73%, no bronchodilator response, Diffusion Capacity 49% predicted • CXR – Bilateral prominent interstitial markings chronic from 4/20/06 comparison, normal heart size

  14. Physical Exam • BP 131/74, P 69, RR 18, SaO2 100% on 2L NC, Afebrile • Gen: A& O x 3, well developed, NAD • HEENT: NC/AT, PERRLA, EOMI, MMM, trachea midline, no thyromegaly or lymphadenopathy • Neck:+JVD, no carotid bruits • Heart: Regular S1S2, no murmur, rubs, or gallops, soft heart sounds

  15. Physical Exam • Lungs:bibasilar crackles, no wheezing or rhonchi • Abdomen: soft, NT/ND, + bowel sounds, no organomegaly, no rebound or guarding • Ext:trace pitting edema BL lower extremities, +2/4 distal pulses, no clubbing or cyanosis • Skin:tightening/shiny skin of lower extremities and hands with mild erythema • Neuro: CN II-XII intact, strength 5/5 BL, cerebellar function intact

  16. Workup • Admitted with decompensated CHF • BNP 3360, mild bilateral pleural effusions and cardiomegaly on CXR, TnI 0.040.080.05 • Pulmonology consult- pHTN unclear etiology consider primary, ANA, RF, Lupus anticoagulant, complete PFT with DLCO, consider right heart catheterization and possible Revatio or Bosantan

  17. Workup • Renal consult for Stage IV renal failure • Recommended discontinue ACE inhibitor, lasix, avoid IV contrast and NSAIDS, testing ordered for autoimmune cause, consider mediastinal LN biopsy to rule out lymphoma/ sarcoidosis

  18. Workup • Normal Esophogram and Upper GI with small bowel follow through • Renal US unremarkable • Renal flow scan showed mild-moderate chronic parenchymal dysfunction bilaterally

  19. Workup • 2D Echo 2/21/2009 - LVEF 60%, moderate to severe PAH with RVSP 64mmHg, LVH, diastolic dysfunction • 2D Echo on 3/25/09 - LVEF 55%, severe PAH with RVSP 60-80mmHg, severely enlarged right atrium/ ventricle, impaired diastolic function

  20. Workup • Cardiology consult • Unable to normalize shortness of breath with diuretics, no obvious heart pathology, pulmonary HTN not generally associated with ETOH toxicity • Rheumatologic workup in process

  21. Workup • ANA Positive, SCL- 70 Ab 154, Negative cold agglutinins, ESR 1, C3 105, C4 11 (low), RF <20, Centromere Ab 12, Histone Abs 19, IgG <1, SSA Ro Ab 15, SSB La Ab 11, Smith Ab 7, Jo-1 Ab 27, DS DNA Ab 69

  22. Summary Our patient: • Positive ANA and SCL-70 Ab • PAH with RVSP of 60-80mmHg on Echo • Raynaud’s phenomenon and thickened mildly erythematous skin changes

  23. Summary • Our patient was treated with supplemental oxygen and was referred to Hospice care due to rapid progression of her symptoms and decline in physical function • She died three months after the diagnosis of scleroderma was made

  24. Discussion

  25. Scleroderma • Mortality rate for systemic sclerosis has been a constant increasing trend for the past several decades [1] • Increased knowledge and understanding of scleroderma may be contributing to the increasing prevalence [1] • Apparent increased mortality of the disease seen from 1999-2002 [1]

  26. Scleroderma • A study by Mendoza showed age-adjusted mortality of systemic sclerosis was 3.2 times greater in women than men [1] • Mortality of this disease continues to trend upward due to the lack of a specifically targeted cure, but improvements in survival have been noted [1] • Most likely explained by decreased morbidity from better management of pulmonary hypertension and renal crisis than in the past [1]

  27. Pulmonary Hypertension • Commonly seen pathologic process and often the cause of death in multiple connective tissue disorders, including systemic sclerosis [2] • Up to 40% of patients with scleroderma have been reported to have pulmonary hypertension [2] • Those with the limited form seem to have an increased risk compared to those with diffuse skin disease [2]

  28. Pulmonary Hypertension • PAH is important in determining the prognosis in patients with systemic sclerosis [2] • Increased risk of developing pulmonary hypertension with positive U3RNP/ fibrillation and anticentromere antibodies [2] • Decrease with antitopoisomerase (Scl-70) antibodies [2]

  29. Raynaud’s and Scleroderma • Diffuse intimal proliferation causes occlusion of the lumen in blood vessels [3] • Leads to the narrowing and vasculopathy seen in pulmonary vascular disease as well as digital vessel pathology (i.e. Raynaud's) [3] • Raynaud's disease has been shown to be an associated risk factor for pulmonary hypertension in patients with systemic sclerosis [3]

  30. Raynaud’s and Scleroderma • Showed that patients with pulmonary hypertension had a longer duration of Raynaud's at the time of diagnosis compared to those without pulmonary hypertension and scleroderma [3] • In the patients with pulmonary hypertension the DLCO was very low at the time of diagnosis [3] • The pulmonary artery systolic pressures were found to be >70mm Hg [3]

  31. Raynaud’s and Scleroderma • Steen found their group of patients with long duration (greater than 10 years) of Raynaud's to have severe pulmonary hypertension with right heart changes • Determined that patients should be suspected to have pulmonary hypertension if they have had longstanding limited disease, ANA or anticentromere, PASP >40 mm Hg or DLCO <65% predicted [3]

  32. Testing Modalities • Echocardiogram, PFTs, exercise testing, and serum biomarkers are among the most common and effective screening modalities for pulmonary hypertension [4] • In symptomatic patients, echocardiography has a 90% sensitivity and 75% specificity for detecting moderate to severe pulmonary artery pressure elevations [4]

  33. Testing Modalities • Several studies have examined exercise echocardiography in patients with scleroderma and reported elevated pulmonary artery pressures [4] • They were not confirmed with right heart catheterization • DLCO in pulmonary function testing was found to show a linear decrease over 15 years in patients with systemic sclerosis who would eventually develop pulmonary hypertension [3]

  34. Testing Modalities • The 6MWD (6 minute walk distance) showed to be useful in assessment of severity of pulmonary hypertension [4] • Has not been used specifically to screen for it in patients with systemic sclerosis • BNP has been used as a marker of right ventricular dysfunction in patients with pulmonary hypertension due to systemic sclerosis [4] • It has not been validated to be a good screening tool alone

  35. Conclusion • This report demonstrates the effects of scleroderma on the lungs demonstrated by rapidly progressing symptoms from pulmonary artery hypertension in our patient • Our patient did complain of pruritis several months prior to her pulmonary symptoms • likely were due to skin changes and tightening secondary to underlying to scleroderma

  36. Conclusion • During her visits with a previous physician she was complaining of pruritis and treated symptomatically with PO antihistamines • Diagnosis of Raynaud’s phenomenon for several years before she presented with pulmonary symptoms • History of alcohol abuse and cardiomyopathy may also have contributed to delay in workup and diagnosis of scleroderma

  37. Conclusion • Although a small percentage of patients with Raynaud’s will end up developing scleroderma, it was an early sign of our patient’s disease • Recognition and complete evaluation would not necessarily have changed the disease course or outcome of our patient • Earlier recognition of the signs and symptoms of scleroderma may have led to an earlier diagnosis in our patient

  38. References • 1. Mendoza F, Derk CT. Systemic Sclerosis Mortality in the United States 1999-2002 Implications for Patient Care. J Clin Rheumatol. 2007;13(4):187-192. • 2. Goldberg A. Pulmonary Arterial Hypertension in Connective Tissue Diseases. Cardiology in Review. 2010;18(2):85-88. • 3. Steen VD. The Lung in Systemic Sclerosis. J Clin Rheumatol. 2005;11(1):40-46. • 4. Bull TM. Screening and therapy of pulmonary hypertension in systemic sclerosis. Curr Opin Rheumatol. 2007;19:598-603.

  39. References • 5. Coghlan JG, Pope J, Denton CP. Assessment of endpoints in pulmonary arterial hypertension associated with connective tissue disease. Curr Opin Pulm Med. 2010;16(suppl 1):S27-S34. • 6. Farmer RG, Gifford RW, Hines EA. Prognostic Significance of Raynaud’s Phenomenon and Other Clinical Characteristics of Systemic Scleroderma. A Study of 271 Cases. Circulation. 1960;XXI:1088-1095.

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