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Secondary Pulmonary Hypertension

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview of Secondary PH/PAH
Antiphospholipid Markers
PH Secondary to MCTD
PH Secondary to Pulmonary Fibrosis
PH Secondary to Scleroderma
PH Secondary to Scleroderma: Dyspnea

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.

Antiphospholipid Antibodies (APLA) Markers in Secondary PH with Scleroderma

Antiphospholipid antibodies are markers for pulmonary hypertension in scleroderma patients.
"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)

Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort. Results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature. The prevalence of PH is much lower than expected from previous studies but confirm the seriousness of the disease complication. Rheumatology (Oxford). 2013 Feb 12.

PH Secondary to Pulmonary Fibrosis

Pulmonary Fibrosis. Pulmonary Hypertension can occur in pulmonary fibrosis, even without severe lung dysfunction or hypoxemia (low oxygen in the blood.) ISN.
Overviews
Mortality/Prognosis
Symptoms
Disease Correlations
Diagnosis
Living with PF
Treatments
Research
Patient Stories
References

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.

PH Secondary to Scleroderma: Dyspnea (Shortness of Breath)

Myositis overlap is another very common cause of shortness of breath in scleroderma patients, besides PH. Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome. A majority of patients with mildly symptomatic systemic sclerosis and pulmonary arterial hypertension in NYHA FC II at diagnosis have a severe disease with poor prognosis. Rheumatology, Feb 2010.
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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