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Respiratory Disorders (Expert Forum)
Scleroderma and Pulmonary Fibrosis
Answered by
This forum is for questions and support regarding lung and respiratory issues such as: Allergies, Asthma, Bronchitis, Colds - Flu, Chronic Cough, COPD, Cystic Fibrosis, Emphysema, Fibrosis, Lung Abscess, Nasal Polyps, Pleurisy, Pneumonia, Sarcoidosis, Sinusitis, Tuberculosis.
Scleroderma and Pulmonary Fibrosis
by amiller920, Jan 16, 2003 12:00AM
Is it possible to have SSc or some other form of Scleroderma w/o having any skin symptoms?
My 69 yo mother in past two years had a constellation of symptoms; so many so that I am hard pressed to believe they all occurred independently. She had dxs of barrett's esophagous, gastric avm's, unexplained diarrhea, an esophageal ulcer, mediastinal adenopathy, "wide open" bronchi as described by pulmonologist on bronchoscopy -- wide open and stiff -- remarkably so according to him.
Lung biopsy results from Hopkins indicated two processes, acute and chronic: acute process manifested as diffuse alveolar damage and focally as infiltrate of PMN cells. Consistent with IPF or collagen vascular disease.
My mother died on 2 Jan 03 of what they were first calling Hamman-Rich syndrome, which I understand to be old term for what is now AIP. Biopsy also shows interstitial fibrosis with honeycome change.
I realize that nothing can change the outcome but I am trying to understand if there is a possibility that this could have been caused by progressive SSc or something related (if so what?). In this way we as a family can be more informed and aware. (Yes, it would have been nice to have done HLA testing prior to her death but that was not done and I didn't put these pieces together until that option was gone). I did get my mother's blood sent to Duke for inclusion in NIH study on familial IPF and called to see if they could do HLA or ANA titers but they said they no longer had the blood.
Any ideas,comments, suggestions, etc, are much appreciated. Tks! Anne
My 69 yo mother in past two years had a constellation of symptoms; so many so that I am hard pressed to believe they all occurred independently. She had dxs of barrett's esophagous, gastric avm's, unexplained diarrhea, an esophageal ulcer, mediastinal adenopathy, "wide open" bronchi as described by pulmonologist on bronchoscopy -- wide open and stiff -- remarkably so according to him.
Lung biopsy results from Hopkins indicated two processes, acute and chronic: acute process manifested as diffuse alveolar damage and focally as infiltrate of PMN cells. Consistent with IPF or collagen vascular disease.
My mother died on 2 Jan 03 of what they were first calling Hamman-Rich syndrome, which I understand to be old term for what is now AIP. Biopsy also shows interstitial fibrosis with honeycome change.
I realize that nothing can change the outcome but I am trying to understand if there is a possibility that this could have been caused by progressive SSc or something related (if so what?). In this way we as a family can be more informed and aware. (Yes, it would have been nice to have done HLA testing prior to her death but that was not done and I didn't put these pieces together until that option was gone). I did get my mother's blood sent to Duke for inclusion in NIH study on familial IPF and called to see if they could do HLA or ANA titers but they said they no longer had the blood.
Any ideas,comments, suggestions, etc, are much appreciated. Tks! Anne
Doctor's Answer
by National Jewish, Jan 20, 2003 12:00AM
, Jan 20, 2003 12:00AM
Yes there are people who have systemic sclerosis (SSc), also called scleroderma, without having any skin symptoms. SSc is a connective tissue disease that causes fibrosis or thickening. This can affect the skin, the joints, and internal organs like the esophagus, intestine, lung, heart, and kidney. When it affects the lungs it’s called interstitial lung disease (ILD). When testing finds no connective tissue disease then the lung problem is called idiopathic pulmonary fibrosis (IPF). This means that the cause is unknown. As you know there is not enough information here to know if a connective tissue disease caused your mother’s ILD.
