Elisabetta Zanatta1, Marie Desportes2, Huy Hoang Do2, Jérôme Avouac3, Andrea Doria4, Antoine Feydy2, Yannick Allanore5. 1. Rhumatologie, Hôpital Cochin, APHP, Université de Paris, Hôpital Cochin, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France; Division of Rheumatology, Department of Medicine-DIMED, University of Padova, 35126 Padova, Italy. 2. Radiologie B, Hôpital Cochin, APHP, Université de Paris, 75014 Paris, France. 3. Rhumatologie, Hôpital Cochin, APHP, Université de Paris, Hôpital Cochin, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France. 4. Division of Rheumatology, Department of Medicine-DIMED, University of Padova, 35126 Padova, Italy. 5. Rhumatologie, Hôpital Cochin, APHP, Université de Paris, Hôpital Cochin, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France. Electronic address: yannick.allanore@aphp.fr.
Abstract
OBJECTIVES: We aimed to clarify the definition, distribution, clinical association and outcomes of large calcinosis in patients with systemic sclerosis (SSc). METHODS: We conducted a systematic literature review (SLR) focusing on SSc-related large calcified masses. Upon updating the terminology and definition, all cases of "pseudotumoral" calcinosis seen at the Cochin and Padova University Hospitals were reviewed. RESULTS: The SLR yielded 30 SSc cases, with large calcified masses mainly defined as "tumoral" or "pseudotumoral". Among the 629 SSc cases included in the Cochin and Padova cohorts, 19 (3%) living and 7 deceased patients were affected by pseudotumoral calcinosis; among these, the great majority had a severe vascular phenotype. The mean age in the whole population (56 cases) was 59 ± 11.4 years with a median disease duration at calcinosis onset of 7 (5-10) years. Twenty-five out of 56 patients (44.6%) had the diffuse cutaneous form of SSc. Anti-topisomerase I and anticentromere were found equally. Pseudotumoral calcinosis were commonly symmetrical and the size ranged from 2 to 15.5 cm. Most patients had multiple site involvement: 52% hand/wrist, 29% shoulders and elbows, 20% hips and 25% spinal calcinosis. Fistulization/ulceration and infections were reported in 32% and 23% of cases, respectively; nerve compression was found in 40% of spinal calcinosis and in one patient with limb calcinosis. There was no clear evidence of clinical and radiological improvement with any treatment. A partial improvement was seen in 7 patients that underwent surgery. CONCLUSION: Pseudotumoral calcinosis may occur in about 3% of SSc patients, commonly symmetrical and in multiple sites without differences regarding the cutaneous subtypes but often in those with a severe vascular phenotype. Medical treatment seems ineffective, whereas a surgical approach may be considered.
OBJECTIVES: We aimed to clarify the definition, distribution, clinical association and outcomes of large calcinosis in patients with systemic sclerosis (SSc). METHODS: We conducted a systematic literature review (SLR) focusing on SSc-related large calcified masses. Upon updating the terminology and definition, all cases of "pseudotumoral" calcinosis seen at the Cochin and Padova University Hospitals were reviewed. RESULTS: The SLR yielded 30 SSc cases, with large calcified masses mainly defined as "tumoral" or "pseudotumoral". Among the 629 SSc cases included in the Cochin and Padova cohorts, 19 (3%) living and 7 deceased patients were affected by pseudotumoral calcinosis; among these, the great majority had a severe vascular phenotype. The mean age in the whole population (56 cases) was 59 ± 11.4 years with a median disease duration at calcinosis onset of 7 (5-10) years. Twenty-five out of 56 patients (44.6%) had the diffuse cutaneous form of SSc. Anti-topisomerase I and anticentromere were found equally. Pseudotumoral calcinosis were commonly symmetrical and the size ranged from 2 to 15.5 cm. Most patients had multiple site involvement: 52% hand/wrist, 29% shoulders and elbows, 20% hips and 25% spinal calcinosis. Fistulization/ulceration and infections were reported in 32% and 23% of cases, respectively; nerve compression was found in 40% of spinal calcinosis and in one patient with limb calcinosis. There was no clear evidence of clinical and radiological improvement with any treatment. A partial improvement was seen in 7 patients that underwent surgery. CONCLUSION:Pseudotumoral calcinosis may occur in about 3% of SSc patients, commonly symmetrical and in multiple sites without differences regarding the cutaneous subtypes but often in those with a severe vascular phenotype. Medical treatment seems ineffective, whereas a surgical approach may be considered.