[A case of overlap syndrome: autoimmune idiopathic hepatitis/pulmonary idiopathic hypertension].
In medical practice we frequently encounter autoimmune syndromes, called "overlap-syndromes," which are of difficult nosographic classification because of the presence of sero-immunologic and clinical features common to various diseases having an autoimmune pathogenesis. Some of these syndromes have already been extensively described in scientific literature such as, for example, the presence of clinical and biohumoral alterations with hepatic and extrahepatic involvement, in the course of viral and autoimmune chronic hepatitis. The described clinical case can be classified as a new "overlap syndrome": Type 1 autoimmune hepatitis (AIH)/Primary pulmonary hypertension (PPH). Although the presence of pulmonary hypertension has been extensively described in the course of various connective tissue diseases (S.L.E., Mixed Connective Tissue Disease, Scleroderma, Hashimoto's Thyroiditis, Sjögren's Syndrome), in recent scientific literature, the association is quite rare. The interest in the described clinical case lies both in the possibility to classify it in the context of a more complex "overlap syndrome" AIH/PPH and in the correlated diagnostic and therapeutic implications. Therefore, in cases of primary pulmonary hypertension, a thorough immunological and hepatic functionality study is always recommended in order to ensure an early diagnosis and a prompt AIH treatment, thus warding off the risk of a rapid progression in cirrhosis.
Bertino
G
G
Dipartimento di Medicina Interna e Patologie Sistemiche Ospedale Santa Marta, Universittà degli Studi di Catania, Italia. gaetanobertino@unict.it
Ardiri
A M
AM
Boemi
P M
PM
Ierna
D
D
Pulvirenti
D
D
Neri
S
S
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001323:Autoantibodies / Q000097:blood; D042241:Early Diagnosis; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D008111:Liver Function Tests; D011241:Prednisone / Q000008:administration & dosage; D013577:Syndrome; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D011241:Prednisone