ERN ReCONNET
Disease Info
Disease: Undifferentiated Connective Tissue Disease
Undifferentiated Connective Tissue Disease (UCTD)
The term UCTD refers to unclassifiable systemic autoimmune diseases that share clinical and serological manifestations with definite connective tissue diseases (CTDs) but not fulfill any of the existing classification criteria. In UCTD, symptoms and autoimmune features are generally milder than in definite CTDs, however major clinical manifestations or objective signs leading to a formal diagnosis of a definite systemic autoimmune disease may later appear in a subset of patients. In others a rather benign, yet disturbing, picture may remain stable for longer. These patients usually fall within the proposed preliminary classification criteria for UCTD that include at least one clinical manifestation of CTDs, positive ANA results, and disease duration of at least three years. In this condition, major organ involvement (such as renal or neurologic) is usually absent, while very frequent symptoms include Raynaudís phenomenon, arthralgias, sicca symptoms, mild haematological abnormalities, alopecia, skin rashes. As such, the therapeutical intervention in UCTD is usually milder than in definite CTD, yet persons suffering from UCTD necessitate a clinical and serological follow-up to individuate the evolution toward a definite CTD or the appraisal of major autoimmune complications requiring more aggressive therapy.
Luca Iaccarino
Luca Iaccarino
Disease Coordinator
Disease Coordinator
Vera Guimarães
Vera Guimarães
ePAG representative
(ad interim)
ePAG representative
(ad interim)
Cristina Pamfil
Cristina Pamfil
Disease Coordinator
Disease Coordinator
List of useful references
- Undifferentiated connective tissue disease: state of the art on clinical practice guidelines
Antunes M, Scirè CA, Talarico R, Alexander T, Avcin T, Belocchi C, Doria A, Franceschini F, Galetti I, Govoni M, Hachulla E, Launay D, Lepri G, Macieira C, Matucci-Cerinic M, Montecucco CM, Moraes-Fontes MF, Mouthon L, Paolino S, Ramoni V, Tani C, Tas SW, Tincani A, Van Vollenhoven R, Zen M, Fonseca J, Bombardieri S, Fonseca JE, Schneider M, Smith V, Cutolo M, Mosca M, Beretta L.
RMD Open. 2019 Feb 26;4(Suppl 1):e000786.
- The diagnosis and classification of undifferentiated connective tissue diseases
Mosca M, Tani C, Vagnani S, Carli L, Bombardieri S.
J Autoimmun. 2014 Feb-Mar;48-49:50-2.
Antunes M, Scirè CA, Talarico R, Alexander T, Avcin T, Belocchi C, Doria A, Franceschini F, Galetti I, Govoni M, Hachulla E, Launay D, Lepri G, Macieira C, Matucci-Cerinic M, Montecucco CM, Moraes-Fontes MF, Mouthon L, Paolino S, Ramoni V, Tani C, Tas SW, Tincani A, Van Vollenhoven R, Zen M, Fonseca J, Bombardieri S, Fonseca JE, Schneider M, Smith V, Cutolo M, Mosca M, Beretta L.
RMD Open. 2019 Feb 26;4(Suppl 1):e000786.
Mosca M, Tani C, Vagnani S, Carli L, Bombardieri S.
J Autoimmun. 2014 Feb-Mar;48-49:50-2.
UCTD centres in ERN ReCONNET
Helsinki University Hospital, Hospital District of Helsinki and Uusimaa, Finland (ReCONNETFIN) (adult and paedriatric)
- Civil Hospital – Brescia
- AOU Careggi, Florence
- AOU Meyer di Firenze (paediatric)
- IRCCS AOU San Martino – Genoa
- Foundation IRCCS CA’Granda Ospedale Maggiore polyclinic – Milan
- ASST Centro Specialistico Ortopedico Traumatologico Gaetano Pini (Presidio Pini)
- AO Padua
- Foundation IRCCS Polyclinic San Matteo, Pavia
- AOU Pisan
- AOU Policlinico Umberto I di Roma
- Fondazione Policlinico Tor Vergata Roma
- A.S.L. Torino – Hub S. Giovanni Bosco
- Azienda Ospedaliera Universitaria Integrata di Verona
- Hospital Clínic de Barcelona y Hospital de Sant Joan de Déu (adult and paediatric)
- Hospital Universitario 12 de Octubre