Welcome to JA and thanks for this question. Im sorry to hear about your sister's diagnosis. Actually NSIP is of two types primarily and these two types are differentiated on the basis of a biopsy. The first type cellular NSIP actually has a very good prognosis and it would usually respond to steroids or another immunosuppressive agent however the other type, fibrotic NSIP is one of the more difficult things to treat as it usually does not respond to most of the medications. Now in her case, given that she was sustained on steroids for a period of 16 months means that the disease is somewhat responsive to the steroids and that is a good sign. Yes she now has water in the lungs (which could be pleural effusions - fluid outside the lungs in the pleural cavity OR pulmonary edema - which is fluid inside the airways of the lungs, usually when we say water on the lungs that indicates pleural effusions which can be managed with various measures like drainage and also be treating the underlying cause), however that water can be managed as i indicated after we determine the cause of this accumulation. This can be done by taking a small sample of the water out from the pleural cavity and analyzing it, it will tell us what the exact cause is and then we can treat it accordingly. In many cases, it is due to the effects of steroids and usually, diuretics would be enough to get rid of the water.
Now as far as her NSIP is concerned, if she is not able to tolerate methotrexate there are several other options that can be used alone or in combination with other medications. All of the following medications have had excellent results in many patients and they have reduced the requirement of steroids significantly. The ones which can be tried include,
- Azathioprine - A common regimen is to start with 25 to 50 mg/day and increase by 50 mg increments every 7 to 14 days up to 1.5 to 2.0 mg/kg per day, but not exceeding a maximum total dose of 200 mg/day.
- Mycophenolate Mofetil (MMF) - The target dose of MMF is generally between 1.5 and 3 g daily, usually in two divided doses (eg, 0.75 to 1.5 twice daily). Starting with lower doses, such as 500 mg twice daily, and increasing to the target dose over three to four months may improve a patient's gastrointestinal tolerance of MMF. This drug has shown excellent efficacy in controlling NSIP in patients especially where methotrexate is not well tolerated.
Now if the above medications dont work or dont help in controlling the disease then it will be labelled as Refractory disease and she can be started on,
- Cyclophosphamide - this is used on a monthly basis and it has been shown to reduce the NSIP symptoms significantly and prevent acute worsening of the disease
- Rituximab - Usually this is used by administering two doses of 1 g each about 1-2 weeks apart and then repeating the dose as necessary
- Calcineurin Inhibitors - These are drugs like cyclosporine or tacrolimus which have been used successfully in managing even the most severe of NSIP cases successfully.
So in your sister's case, there are still a number of treatment options available which have not been tried as of yet and I think given her situation and the fact that it has been just two years since she was started on steroids and the disease has not progressed to a severe level as of yet, I think she can be started on azathioprine or mycophenolate right now and then we can adjust her treatment according to the response.
A lung transplant is certainly an option which is available however we usually dont consider a lung transplant until the lungs have been severely damaged by the NSIP, or atleast until the NSIP has placed some strain on the heart and caused the development of pulmonary hypertension. A lung transplant is the last resort measure in situations like these which is why i think at the moment we can wait and try the other medications that I have mentioned above.
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