Dinesh Khanna, MD, Professor of Rheumatology and Medicine at the University of Michigan, discusses diffuse cutaneous systemic sclerosis (dcSSc) and how these patients are typically treated.
dcSSc is a subtype of systemic scleroderma (also known as systemic sclerosis) that is characterized by fibrosis and affects multiple body systems. The disease can occur at any age but most often affects women between 30 and 50 years of age. Symptoms include Raynaud’s phenomenon; skin fibrosis beginning on the fingers and face that rapidly becomes generalized; telangiectasias on the thorax, face, lips, tongue, and fingers; gastroesophageal reflux; and dysphagia along with weight loss, vomiting, diarrhea or constipation. Dry mouth and dental involvement can occur. Joint pain, muscular pain, weakness, cramps, and destruction of the tips of the fingers or toes are frequent. Severe lung problems (e.g., pulmonary arterial hypertension, interstitial lung disease) may occur, as well as kidney problems. The exact cause of the condition is unknown.
As Dr. Khanna explains, there is currently no approved targeted therapy for dcSSc. As such, management of these patients is dependent on specific symptoms rather than prevention. Dr. Khanna mentions the use of immunosuppressive therapies to reduce inflammation and fibrosis. Anti-inflammatory drugs may also be used to reduce inflammation as well as help with pain and/or swelling, and vasodilators may help with Raynaud’s phenomenon.
Fortunately, Horizon Therapeutics recently recruited the first patient in their phase 2b clinical trial of HZN-825, an investigational targeted therapy for dcSSc.
To learn more about dcSSc and other autoimmune conditions, visit checkrare.com/diseases/autoimmune/
