Today is the World Scleroderma Day, a day set aside to increase the sensitization on a rare autoimmune condition, the theme for the year is Unmet clinical needs in Scleroderma, the PDC Crew had a chat with Dr. Olaosebikan B. Hakeem Consultant Rheumatologist/Physician at the Lagos State University Teaching Hospital (LASUTH), Ikeja. The graduate of the University of Aberdeen, Institute of Applied Health Science and co-author of Building a Rheumatology Team for Africa: Enhancing the roles of patients and non-specialists gives an insight into the world of Scleroderma and its treatment. Excerpts
PDC: Kindly explain to us what Scleroderma is?
BHO: Let me start by breaking scleroderma down- Sclero means HARD while derma is the SKIN. It is an autoimmune connective tissue diseases in which body produces excess proteins called antibodies that attack the skin and internal organs. The skin becomes thickened and hard due to excessive collagen production and deposition in skin as a response to antibody attack. However, scleroderma is not a contagious or infectious disease.
PDC: As a disease connected with an overactive immune system, what are the symptoms of the disease?
BHO: The symptoms vary from patient to patient. Some may have mild symptoms with just skin problems while others may have skin problems with life threatening internal organs involvement. Occasionally, there may not be skin problem but patient has internal organs affectations.
Some of the earliest symptoms include- Raynaud’s symptoms- which is the increase sensitivity- finger colour changes on exposure to cold; Hand and feet swelling; Sclerodactyly- thickened skin of the fingers or toes.
While late symptoms include- thickened skin of the trunk, face, extremities; reduced oral aperture, area of dark and light skin; itching of the skin. Others are dryness of the skin, mouth and eyes; Finger ulcers or wounds and scar tissues in the lung, heart, stomach etc. The Kidney can also be affected which is called Scleroderma Renal Crisis.
PDC: What are the causes of the disease?
BHO: Causes of Scleroderma are not unknown but certain factors can increase the chance of having this condition for example: family history of autoimmune diseases, female sex, ethnicity and exposure to mineral oils, silica, some drugs, petroleum products and some chemicals. It also affects children but more common in adult than children.
PDC: What are the various types of Scleroderma?
BHO: Yes, there are two major types- Localised and Systemic Scleroderma. The localised type affects specific part of the skin but does not affect internal organs and it is more common in children while Systemic Scleroderma affects the skin and internal organs. It is also subdivided into two- Limited and Diffused based on distribution of the thickened skin and organ affected.
PDC: Is the condition more prevalent in any particular gender?
BHO: Scleroderma is commoner in female than male and common among people between the age group of 35 to 55 years but this does not say that it cannot affect any age group can be affected.
PDC: With all the aforementioned symptoms, what is the treatment of this disease?
BHO: No single treatment for all patients and treatment is usually based on symptoms or the organ affected. However some treatments such as Skin softening cream for the skin, drug for the skin itching and dryness, medications, drugs for Raynaud’s, drugs for lung diseases and pulmonary hypertension etc.
PDC: The disease is not as prevalent like Hepatitis, AIDS and other known names, is it prevalent in Nigeria also?
BHO: It is a rare disease worldwide but commoner among African Americans. It is also becoming increasingly diagnosed in Nigeria as we have published an article in reputable journal on the earliest cases seen as well as the upsurge in the new cases of Scleroderma patients attending clinics in both Lagos University Teaching Hospital (LUTH) and Lagos State University Teaching Hospital (LASUTH).
PDC: As a disease that has unique manifestations for individuals, what are the various investigations required to ascertain that the patient is really affected by scleroderma?
BHO: No single investigation is adequate to make diagnosis as the disease can be diagnosed without investigation but we use investigation to identify internal organs that are involved and to determine severity of Scleroderma. Some of the investigations include Chest x-ray, Pulmonary Function test, Electrocardiogram, echocardiogram, antibody test (antiscl70, anticentromere and antiRNA PolymeraseIII)
PDC: What advice do you have for patients managing the condition?
BHO: Just like any other chronic disease like hypertension, diabetes, scleroderma has no proven cure but there are effective treatment to control symptoms and reduce progression of organ damage. Before the advent of effective treatment, it used to be a death sentence as patients died from kidney crisis. They should not allow scleroderma to define them and that is why we say, Scleroderma will not take my smile.
They should seek medical attention as early as possible when they notice any of the above symptoms. They should avoid excessive sunlight exposure. They should avoid exposure of fingers or toes to cold. As we are aware of unmet clinical and research needs in Scleroderma, we call on Governement, Private organizations and other stakeholders to support the research on Scleroderma