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Scleroderma with ILD
Scleroderma is a rare multisystem autoimmune disease that affects the skin and internal organs, including lungs, which can also lead to interstitial lung disease (ILD)1
What is scleroderma with interstitial lung disease (scleroderma with ILD)?
Scleroderma is a rare condition that affects 0.6-2.3 and 1.4-5.6 people per 100,000 each year in Europe and North America, respectively.2 Lung involvement by scleroderma is very common, either involving the blood vessels in the lung or the supporting structures (interstitium) of the lung leading to interstitial lung disease (ILD).3 At least a third to a half of scleroderma patients have symptoms from ILD but more sophisticated testing can find evidence of ILD in almost all patients with the disease.
ILD can be associated with scarring in the lungs. This lung (pulmonary) scarring (fibrosis), occurs between the oxygen containing sacs in the lung and the blood vessels that carry oxygen to the rest of the body. The scarring pulls on the airways in the lung causing cough, which is often the first symptom, and makes it harder for oxygen to get into the body, causing shortness of breath.4,5
La mayoría de las personas no advierten los cambios en sus pulmones al principio y posiblemente piensen que no están en forma o están cansadas. Esto puede ser verdad, pero es importante conocer nuestro cuerpo y advertir los cambios.
Mientras más pronto se detecte la afectación pulmonar, más pronto podrá someterse a pruebas y obtener apoyo, dado que el médico puede decidir la mejor manera de proceder en su caso.
Los síntomas de esclerodermia con EPI pueden incluir los siguientes:4,6
Sensación de mucho cansancio durante el transcurso de un día normal.
Falta de aire al realizar tareas simples como subir escaleras.
Tos seca que no desaparece.
Dificultad para respirar profundamente.
Hay una variedad de síntomas que puede presentar con la esclerodermia con fibrosis pulmonar y algunos de estos empeorarán con la progresión de su enfermedad. Tenga en cuenta que hay cambios en el estilo de vida y diferentes tratamientos que pueden ayudarle a controlarlos. Los síntomas también pueden variar de una persona a otra, por lo que sus síntomas podrían no ser los mismos que los de otra persona que padece de fibrosis pulmonar.
“I first noticed it at work – I got out of breath if I walked up the stairs”
Monitoring symptoms
Al diagnosticar la esclerodermia con EPI, su equipo de tratamiento evaluará muchas áreas de su salud. Por ejemplo, le preguntarán sobre sus antecedentes laborales, antecedentes médicos familiares, algún posible uso de medicamentos, y si alguna vez ha fumado.
También se le hará un examen físico para que el médico comprenda mejor su salud pulmonar. No obstante, es posible que se necesiten otras pruebas para obtener un diagnóstico claro.4
La esclerodermia con EPI puede progresar, lo que significa que podría empeorar con el tiempo. Sin embargo, no hay manera de predecir si esto sucederá ni con qué rapidez lo hará. Es posible que algunos pacientes no presenten cambios en sus síntomas, en tanto que otros pacientes podrían experimentar un empeoramiento más rápido de sus síntomas. Se desconoce por qué la enfermedad de algunas personas evoluciona más rápido que la de otras.7
Talk to your doctor about treatment options for scleroderma with ILD. There are medications to help treat lung involvement caused by scleroderma. They will be best able to provide guidance and support tailored to your individual situation and suggest the best way moving forward
Your mental wellbeing
Your wellbeing
Some days may be tougher than others. Regardless of how you’re feeling we can point you in the direction of help.
Other scleroderma life hacks
If you have scleroderma, daily life can be challenging, both practically and mentally. We can help you with a few easy life hacks helping you get on top of your mental health, holiday preparation and childcare.
Cold hacks
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The cold weather can be challenging if you have scleroderma. But watch Annelise explain some tips that can make your day easier, including a hand warming trick using rice, a sock and a microwave.
Kitchen hacks
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Scleroderma can mean a few challenges in the kitchen.... Edith gives us a few handy tips that help her, including how to use hair rollers to help grip cutlery.
Mental health hacks
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Nathan is a counsellor, working in partnership with people living with chronic disease. He explains how to express any difficult feelings you have and ways to help yourself out of a low mood.
Holiday hacks
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Preparing for a big family gathering can be tiring. Watch to see how Joyce does it, saving precious energy and overcoming dexterity issues to wrap beautiful gifts.
Childcare hacks
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Looking after a family can be even more of a challenge with Scleroderma, but Natalie shows how a little creative thinking can help with everything from changing diapers to getting the kids out of the door with less fuss.
Key takeaways
Scleroderma is a rare condition. ILD can occur in patients with scleroderma, which means that the lung tissue might get scarred, making it harder for oxygen to enter the blood resulting in shortness of breath
When looking for the correct diagnosis, your treatment team may perform several tests to get a good understanding of your lung health
Scleroderma with ILD can get worse over time but it is not known why some people’s disease progresses faster than others
Hudson M, Lo E, Lu Y, et al. Cigarette smoking in patients with systemic sclerosis. Arthritis Rheum. 2011;63(1):230–238.
Bergamasco A, Hartmann N, Wallace L, Verpillat P. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol. 2019;11:257–273.
Olson A, Gifford A, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev. 2018;27(150):180077.
British Lung Foundation. Pulmonary Fibrosis. Available at: https://www.blf.org.uk/support-for-you/pulmonary-fibrosis [Accessed April 2019].
Cappelli S, Bellando Randone S, et al. Interstitial lung disease in systemic sclerosis: where do we stand? Eur Respir Rev. 2015;24(137):411–419.
NHS. Scleroderma. Available from: https://www.nhs.uk/conditions/scleroderma/ [Accessed March 2022].
Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254.
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