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Suggest Treatment For Systemic Sclerosis
Question: Hi My wife was diagnosed with Systemic Sclerosis and now has affected lungs..what is the way forward
Brief Answer:
Pulmonary hypertension and lung fibrosis are common complications.
Detailed Answer:
Thanks for your question on Healthcare Magic.
I can understand your concern.
Systemic sclerosis causes following pulmonary complications.
1. Interstitial lung disease (ILD)
2. Pulmonary Hypertension (PH).
3. Pleural involvement
4. Alveolar heamorrhage
5. Malignancy
6. Aspiration pneumonia
7. Spontaneous pneumothorax etc.
Amongst all, ILD and PH are most common.
So regular 6 monthly PFT, 2d echo and HRCT (high resolution CT) thorax are needed.
Control of systemic sclerosis through steroids and other drugs is must.
For ILD, perfinex (perfinodone) should be started.
For PH, bosentan or tadalafil should be started.
Incentive spirometry, adult pulmonary vaccines are needed to prevent recurrent respiratory tract infection and improve lung functions.
Hope I have solved your query. I will be happy to help you further. Wishing good health to your wife. Thanks.
Pulmonary hypertension and lung fibrosis are common complications.
Detailed Answer:
Thanks for your question on Healthcare Magic.
I can understand your concern.
Systemic sclerosis causes following pulmonary complications.
1. Interstitial lung disease (ILD)
2. Pulmonary Hypertension (PH).
3. Pleural involvement
4. Alveolar heamorrhage
5. Malignancy
6. Aspiration pneumonia
7. Spontaneous pneumothorax etc.
Amongst all, ILD and PH are most common.
So regular 6 monthly PFT, 2d echo and HRCT (high resolution CT) thorax are needed.
Control of systemic sclerosis through steroids and other drugs is must.
For ILD, perfinex (perfinodone) should be started.
For PH, bosentan or tadalafil should be started.
Incentive spirometry, adult pulmonary vaccines are needed to prevent recurrent respiratory tract infection and improve lung functions.
Hope I have solved your query. I will be happy to help you further. Wishing good health to your wife. Thanks.
Above answer was peer-reviewed by :
Dr. Yogesh D
reports attached
Dear Dr. Bhavsar,
Thank you for the answer and your wishes.
As per the reports she has ILD with UIP pattern..
1. If you could advise what this is .
2. I know it is a progressive disease and the comparison report shows mild increase in the disease in space of 3 months. How will it affect the quality of life moving ahead
3. What is the cure / options that i have.
I am a little worried and would like to know the options to keep myself ready financially as well.
Dear Dr. Bhavsar,
Thank you for the answer and your wishes.
As per the reports she has ILD with UIP pattern..
1. If you could advise what this is .
2. I know it is a progressive disease and the comparison report shows mild increase in the disease in space of 3 months. How will it affect the quality of life moving ahead
3. What is the cure / options that i have.
I am a little worried and would like to know the options to keep myself ready financially as well.
Brief Answer:
Please let me know her symptoms.
Detailed Answer:
Thanks for your follow up question on Healthcare Magic.
I can understand your concern.
I have gone through the reports you have attached.
UIP is Usual Interstitial Pneumonia.
It is type of ILD which is more common.
In this, there is destruction of lung interstitial tissue and formation of honeycombing.
This honeycombing does not take part in gas exchange. So practically speaking honeycombing is a dead tissue.
Since ILD is progressive disease, honeycombing a dead tissue also progress and occupy more and more lung. So over a period of time, patient deteriorates.
So patient worsens in th form of symptoms like breathlessness, cough, chest pain, edema feet, palpitations, hypoxia etc.
So patient needs life long oxygen support and treatment for ILD (perfinex) and its complications (Pulmonary hypertension, Heart failure, arrhythmia etc).
So may I know her symptoms?
I will be happy to help you further. Wishing good health to your wife. Thanks.
Please let me know her symptoms.
Detailed Answer:
Thanks for your follow up question on Healthcare Magic.
I can understand your concern.
I have gone through the reports you have attached.
UIP is Usual Interstitial Pneumonia.
It is type of ILD which is more common.
In this, there is destruction of lung interstitial tissue and formation of honeycombing.
This honeycombing does not take part in gas exchange. So practically speaking honeycombing is a dead tissue.
Since ILD is progressive disease, honeycombing a dead tissue also progress and occupy more and more lung. So over a period of time, patient deteriorates.
So patient worsens in th form of symptoms like breathlessness, cough, chest pain, edema feet, palpitations, hypoxia etc.
So patient needs life long oxygen support and treatment for ILD (perfinex) and its complications (Pulmonary hypertension, Heart failure, arrhythmia etc).
So may I know her symptoms?
I will be happy to help you further. Wishing good health to your wife. Thanks.
Above answer was peer-reviewed by :
Dr. Kampana
Thank you.
She has had Systemic Sclerosis for about 5 years and taking medications for the same. Lungs ..we got to know about 6 months back
As such she gets tired by end of day after household work, bit of breathless not much. She has had this cough dry for quite some time (years) and when she starts coughing it goes on for about 10-15 seconds or so non stop.
Her fingers turn blue the moment she gets into a colder zone.
No chest pain,edema feet or palpitations so far.
She has had Systemic Sclerosis for about 5 years and taking medications for the same. Lungs ..we got to know about 6 months back
As such she gets tired by end of day after household work, bit of breathless not much. She has had this cough dry for quite some time (years) and when she starts coughing it goes on for about 10-15 seconds or so non stop.
Her fingers turn blue the moment she gets into a colder zone.
No chest pain,edema feet or palpitations so far.
Brief Answer:
She has started symptoms of ILD.
Detailed Answer:
Thanks for your follow up question on Healthcare Magic.
I can understand your concern.
So she has already started symptoms of ILD.
Please let me know
1. PFT done or not?
2. 2d echo done or not?
PFT is must to know about lung functions.
2d echo is must to rule out pulmonary hypertension and other cardiac complications.
So please let me know if PFT and 2d echo are done or not.
I will be happy to help you further. Wishing good health to your wife. Thanks.
She has started symptoms of ILD.
Detailed Answer:
Thanks for your follow up question on Healthcare Magic.
I can understand your concern.
So she has already started symptoms of ILD.
Please let me know
1. PFT done or not?
2. 2d echo done or not?
PFT is must to know about lung functions.
2d echo is must to rule out pulmonary hypertension and other cardiac complications.
So please let me know if PFT and 2d echo are done or not.
I will be happy to help you further. Wishing good health to your wife. Thanks.
Above answer was peer-reviewed by :
Dr. Kampana
Thank you, attaching reports
Brief Answer:
She is having severe restriction on PFT.
Detailed Answer:
Thanks for your follow up question on Healthcare Magic.
I can understand your concern.
I have gone through the reports you have attached.
Her 2d echo is normal. So no pulmonary hypertension or other cardiac complications.
But her PFT report is showing severe restrictive defect.
And both these reports are almost 1 year old.
So better to get done fresh PFT and 2d echo to know the progression of disease.
May I know on which medicines she is at present?
What is her baseline Spo2?
Please reply me answers of above asked questions, so that I can guide you better. I will be happy to help you further. Wishing good health to your wife. Thanks.
She is having severe restriction on PFT.
Detailed Answer:
Thanks for your follow up question on Healthcare Magic.
I can understand your concern.
I have gone through the reports you have attached.
Her 2d echo is normal. So no pulmonary hypertension or other cardiac complications.
But her PFT report is showing severe restrictive defect.
And both these reports are almost 1 year old.
So better to get done fresh PFT and 2d echo to know the progression of disease.
May I know on which medicines she is at present?
What is her baseline Spo2?
Please reply me answers of above asked questions, so that I can guide you better. I will be happy to help you further. Wishing good health to your wife. Thanks.
Above answer was peer-reviewed by :
Dr. Raju A.T
Answered by
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