| Pulmonary Hypertension |
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Secondary Pulmonary Hypertension |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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Overview of Secondary Pulmonary Hypertension |
| Treatments— and their effectiveness—can vary depending on whether PH is primary or secondary. |
When PH occurs along with other lung, heart, or systemic connective tissue disease (such as scleroderma), it is called Secondary Pulmonary Hypertension. The distinction is important because treatments, and their effectiveness, can vary depending upon whether the pulmonary hypertension is primary or secondary. (Also see: Isolated Pulmonary Hypertension) |
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| Is Pulmonary Arterial Hypertension Really a Late Complication of Systemic Sclerosis? In contrast to the expected scenario, early onset PAH occurred in approximately half of SSc patients. Early onset PAH was as frequent among patients with diffuse as limited SSc. Annual screening for PAH should be implemented immediately after SSc diagnosis for all patients. Eric Hachulla, MD, PhD Chest May 2009. |
| Risk Factors Associated with Pulmonary Arterial Hypertension (PAH) in Colombian Patients with Systemic Sclerosis (SSc): Review of the Literature. PAH may be a frequent complication of SSc in the Colombian population regardless of disease subtype. J Rheumatol 2008;35:244-50 |
Antiphospholipid Antibodies (APLA) Markers in Secondary PH with Scleroderma |
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| "Listen to the Patient" Anticoagulation Is Critical in the Antiphospholipid (Hughes) Syndrome (APS). Affecting, almost uniquely, both veins and arteries, the clinical features (of APS) range through deep vein thrombosis (DVT), chronic leg ulcers, recurrent miscarriages, headache, heart attacks, renal vein and artery thrombosis, to pulmonary embolism and even pulmonary hypertension. The Journal of Rheumatology. (Also see: Antiphospholipid Syndrome, Leg Ulcers, Pregnancy and Scleroderma, and Cardiac Involvement) |
PH Secondary to Mixed Connective Tissue Disease (MCTD) |
| Pulmonary Arterial Hypertension (PAH) Complicating Connective Tissue Diseases (CTD). Treatment strategies for PAH associated with CTD are similar to but are distinctly less effective than those for idiopathic PAH. PM Hassoun. Semin Respir Crit Care Med. 2009 Aug;30(4):429-39. |
PH Secondary to Pulmonary Fibrosis |
| PH can occur in pulmonary fibrosis, even without severe lung dysfunction or hypoxemia (low oxygen in the blood.) |
PH Secondary to Systemic Sclerosis (SSc, or Scleroderma) |
| Prediction Of Pulmonary Hypertension Related To Systemic Sclerosis (SSc) By An Index Based On Simple Clinical Observations. Using routine clinical observations, we developed a simple score, which accurately predicted the risk of PH in SSc. C. Meune. EULAR 2011 OPO196. Ann Rheum Dis 2011;70(Suppl3):135. |
| PH (Pulmonary Hypertension) and Scleroderma. In scleroderma, 10–15% of patients have evidence of pulmonary arterial hypertension and another 10–15% of patients have pulmonary hypertension as a complication of interstitial lung disease and/or left ventricular dysfunction. Pulmonary Hypertension Society. 2010. |
| Is Pulmonary Arterial Hypertension (PAH) Really a Late Complication of Systemic Sclerosis (SSc)? In contrast to the expected scenario, early onset PAH occurred in approximately half of SSc patients. Early onset PAH was as frequent among patients with diffuse as limited SSc. Annual screening for PAH should be implemented immediately after SSc diagnosis for all patients. Eric Hachulla, MD, PhD. Chest. October 2009. |
| Peripheral Arterial Stiffness and Endothelial Dysfunction in Idiopathic and Scleroderma Associated Pulmonary Arterial Hypertension. Our study shows a trend towards increased arterial stiffness in scleroderma (nonsignificant), and also peripheral endothelial dysfunction in idiopathic-PAH and in scleroderma-PAH. These findings suggest involvement of different vessels in scleroderma-PAH compared to idiopathic-PAH. Nir Peled JRheum. April 2009, 36 (4). |
| Predictors of Intrinsic Pulmonary Arterial Hypertension (IPAH) in Patients with Limited Cutaneous Systemic Sclerosis (lcSSc). Limited scleroderma (LcSSc) patients with onset of Raynaud's phenomenon at an older age, low DLCO (carbon monoxide diffusing capacity) and/or history of scleroderma renal crisis should be regarded as having an increased risk of developing IPAH and should be monitored closely for this complication. Tatiana Rodriguez-Reyna. 1861/533 ACR 2006. (Also see: Types of Scleroderma, Limited Scleroderma, Kidney Involvement, and Raynaud's) |
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PH Secondary to Scleroderma: Dyspnea (Shortness of Breath) |
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| Pulmonary fibrosis is a frequent cause of PH in scleroderma patients. |
Diagnosis and Management of Pulmonary Hypertension in Systemic Sclerosis. Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH) caused by pulmonary arterial hypertension (PAH), left ventricular disease, or pulmonary fibrosis. Prognosis and therapeutic response are worse in PAH-SSc than in other PAH categories (median survival, 1–3 yr). Current Rheumatology Reports, Volume 12, Number 1 / February, 2010. (Also see: Cardiac Involvement and Pulmonary Fibrosis) |
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