Clinical Snapshot
Telangiectasias as a Forerunner of Pulmonary Hypertension
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A retired female patient reported gradually progressive dyspnea and a dry cough. After ruling out primary cardiac causes, plethysmography revealed a marked reduction in CO diffusion capacity (28% of predicted). From a pulmonological perspective, an initial diagnosis of precapillary pulmonary hypertension and interstitial lung disease was made. Home oxygen therapy was initiated. A rheumatological evaluation showed positive anti-centromere (CENP-A/-B) antibodies, which are associated with limited cutaneous systemic sclerosis (lcSSc). Given the absence of sclerodactyly and late organ involvement, further disease manifestations were sought to confirm the diagnosis. On examination, extensive cutaneous telangiectasias on the back were observed (a); when asked, the patient reported that these had been present in that form for 30 years. Based on the acronym CREST (calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, telangiectasias), which summarizes common lcSSc symptoms, it is possible to identify long-standing Raynaud’s syndrome as well as reflux disease. Nailfold capillary microscopy confirmed the presence of vasculopathy (b). With immunomodulatory therapy (prednisolone, azathioprine, and most recently, mycophenolate mofetil) and vasoactive treatment (tadalafil), dyspnea improved over the course of treatment. The skin findings, which ultimately preceded the pulmonary manifestations by decades, remained unchanged.
Fabian Thomas Hermann Ullrich, Dr. med. Matthias Bernhard Thaler, Prof. Dr. med. Hendrik Schulze-Koops, Klinikum der Universität München, LMU Klinikum, Medizinische Klinik und Poliklinik IV, Sektion Rheumatologie und Klinische Immunologie, fabian.ullrich@med.uni-muenchen.de
Conflict of interest statement: The authors state that no conflict of interest exists.
Translated from the original German by Christine Rye.
Cite this as: Ullrich FTH, Thaler MB, Schulze-Koops H: Telangiectasias as a forerunner of pulmonary hypertension. Dtsch Arztebl Int 2025; 122: 507. DOI: 10.3238/arztebl.m2025.0118
