Think before: a guide to early diagnosis of pulmonary hypertension in patients with connective tissue diseases and vice versa.

Connective Tissue Diseases (CTDs) is a group of autoimmune disorders with common clinical and serological characteristics. Among them, Systemic sclerosis (SSc) is the disease most often associated with Pulmonary Arterial Hypertension (PAH), which is often diagnosed with delay for the unspecific symptomatology. A delayed diagnosis of PAH in patients with SSc may affect prognosis, but also a missed recognition of CTD in patients with PAH may be detrimental for clinical management. This statement produced by a working group made up of rheumatologists and pneumologists has the objectives to suggest items useful for the identification, diagnosis and classification of CTDs in cases of already identified pulmonary disease and how to differentiate signs and symptoms arising respectively from parenchymal and vascular diseases in order to define a set of proposed recommendations on which consensus may be reached for their application in clinical practice.