| LUNG (PULMONARY): MAIN MENU |
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| Pulmonary Hypertension: MAIN MENU |
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| Secondary Pulmonary Hypertension |
| This page was written by Shelley Ensz, and has not been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See 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) | |
| 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 VOLUME 30: NO. 4 APRIL 2003. (Also see: Antiphospholipid Syndrome, Leg Ulcers, Pregnancy and Scleroderma, and Cardiac Involvement) |
| PH Secondary to Mixed Connective Tissue Disease (MCTD) |
| Pulmonary Arterial Hypertension in Mixed Connective Tissue Disease: Clinical Abnormalities and Successful Treatment with Prostacyclin. Combination treatment of prostacyclin, corticosteroids and immunosuppressive agents may effectively control and improve cardiac function in PAH underlying MCTD. J. Végh. AB0113 EULAR 2004. (Also see: Mixed Connective Tissue Disease) |
| PH Secondary to Pulmonary Fibrosis |
| PH can occur in pulmonary fibrosis, even without severe lung dysfunction or hypoxemia (low oxygen in the blood.) | Pulmonary Hypertension (PH) in Patients With Interstitial Lung Diseases. PH in patients with interstitial lung diseases (ILDs) is not well recognized and can occur in the absence of advanced pulmonary dysfunction or hypoxemia. The ILDs most commonly associated with PH include connective tissue disease-related ILD, sarcoidosis, idiopathic pulmonary fibrosis, and pulmonary Langerhans cell histiocytosis. Red Orbit. 04/13/07. (Also see: Pulmonary Fibrosis and Isolated Pulmonary Hypertension) | |
| PH Secondary to Systemic Sclerosis (SSc, or Scleroderma) |
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| 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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| Lung lesion is a most common organ change in systemic scleroderma (SSD) detectable in approximately 70% of the patients at autopsy. This study has provided evidence that echocardiography is of high informative value in detecting pulmonary hypertension (PH) and right cardiac changes in patients with systemic scleroderma, which shows this technique to be a valuable screening in these cases. PubMed. Klin Med (Mosk). 2004;82(5):47-50. (Also see: Cardiac Involvement) |
| PH Secondary to Scleroderma: Dyspnea (Shortness of Breath) |
| Myositis overlap is another very common cause of shortness of breath in scleroderma patients, besides PH. | Dyspnoea in Systemic Sclerosis. Diffuse systemic sclerosis/myositis overlap is a known entity. Anti PmScl antibodies are seen in 40-50% of patients and are associated with positive HLA-DR3. Consider muscle disease as the cause for dyspnoea and dysphagia in systemic sclerosis. N. R. Priddee. SAT0228 EULAR 2006. (Also see: Dermatomyositis and Antibodies) | |
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