The risk of blindness, due to acute ischemic ocular events, is the most feared complication of giant cell arteritis (GCA) since the middle of the 20th century. A decrease of its rate has occurred after the advent of corticoid therapy for this vasculitis, but it seems to have stabilized since then. Early diagnosis and treatment of GCA is key to reducing its ocular morbidity. However, it is not uncommon for ophthalmological manifestations to inaugurate the disease, and the biological inflammatory reaction may be mild, making its diagnosis more challenging. In recent years, vascular imaging has opened up new possibilities for the rapid diagnosis of GCA, and ultrasound has taken a central place in fast-track diagnostic processes. Corticosteroid therapy remains the cornerstone of treatment and must begin immediately in patients with visual symptoms and suspicion of GCA. In that situation, the administration route of corticotherapy, intravenous or oral, is less important than its speed of delivery, any hour of delay worsening the prognosis.
Tumour necrosis factor-α [TNF-α] inhibitors have revolutionised the management of chronic inflammatory conditions. A number of cutaneous adverse events have been reported with TNF inhibition, including vasculitis. Most reactions are mild and rarely warrant treatment withdrawal. Here we describe a patient with Crohn’s disease treated with adalimumab in whom severe multivisceral Henoch-Schönlein purpura developed, including neurological involvement, requiring definitive TNF blocker withdrawal.
ObjectiveIschemic digital ulcers (DUs) are frequent and severe complications of systemic sclerosis (SSc). Treatment options for SSc-related digital vasculopathy are based on aggressive vasodilation, with the objective to improve blood flow in ischemic areas. Intravenous prostanoids are recommended to treat active DUs. However, the level of evidence for the duration of 5 days is low. Therefore, the aim of this study was to determine whether prolonging the infusion beyond 5 days increases the rate of healing of active DUs in SSc.MethodsThis is an observational longitudinal retrospective bicenter study from 2000 to 2017. The objective was to compare the healing rate and time (defined by a healing of at least 50% of DUs) between two durations of iloprost administration: 5 days or less, or more than 5 days.ResultsForty-one patients, with a mean age of 47 ± 15 years at diagnosis and 32 (78%) females have been included. Systemic sclerosis was diffuse in 10 (24%) cases and 13 (32%) had an interstitial lung disease. A total of 243 iloprost infusions for DUs were performed: 140 infusions for 5 days or less, and 103 infusions for more than 5 days (prolonged duration). Patients with active DUs which received >5 days of iloprost had higher modified Rodnan skin scale at the time of iloprost infusion (median 33 vs. 15; p < 0.05), more interstitial lung disease (44 vs. 27%; p < 0.05), more anti-topoisomerase I antibody positivity (59 vs. 44%; p < 0.05), and received more previous cyclophosphamide therapy (48 vs. 19%; p < 0.05). While the number of active DUs before iloprost infusion was not significantly different among those who received ≤5 days and >5 days of iloprost, the time to healing after iloprost infusion significantly decreased in SSc patients who received >5 days iloprost infusion: 48 [7–392] vs. 91 [9–365] days (p < 0.05). The proportion of SSc patients with healed DUs tended to increase in patients with >5 days iloprost infusion (log rank = 0.06). The number of patients with complete DU healing at day 90 was significantly increased in SSc who received >5 days of iloprost: 53 (51%) vs. 52 (37%) (p < 0.05). In addition, the time to healing was not significantly associated with the use of calcium channel blockers, endothelin receptor antagonists or a combination of PDE-5 inhibitors.ConclusionProlonging duration of iloprost >5 days could improve the healing rate and the time to healing of SSc-related DUs. Prospective randomized studies are needed to confirm these data and define the optimal duration of iloprost therapy.
Background:Non-Hodgkin B-cell lymphoma (NHL), especially mucosa-associated lymphoid tissue (MALT) lymphoma, is one of the main complications of primary Sjögren’s syndrome (pSS). Frequent extranodal lymphoproliferation makes its diagnosis challenging and obtaining a biopsy difficult. Since pSS-associated lymphomas are very frequently MALT lymphomas with salivary gland involvement, we hypothesized that minor salivary gland biopsy (MSGB) could be useful for NHL diagnosis in this context.Objectives:To evaluate the potential contribution of MSGB for the diagnosis of pSS-associated MALT lymphoma by comparing patients diagnosed with NHL based on MSGB or another tissue.Methods:All pSS patients (ACR/EULAR 2016 classification criteria), from the Paris National Referral Centers for Rare Systemic Autoimmune Diseases, diagnosed with NHL between January 2010 and October 2019, were included. Each patient’s clinical, biological, radiological and therapeutic information was collected retrospectively at NHL diagnosis and 1-year later. Only patients with MSGB available were analyzed; they were divided into 2 groups according to MSGB results for NHL: MSGB+and MSGB–.Results:Among 36 pSS patients diagnosed with NHL during the study period, 25 had an MSGB available at the time of NHL diagnosis. Among them, 13 MSGBs contained NHL (MSGB+). MSGB was the only site enabling NHL diagnosis for 10/13 (77%); pSS and NHL were diagnosed simultaneously in 4/13 (31%). MSBGs were NHL–for lymphomas diagnosed based on other tissue samples for 12 (48%) patients (MSGB–). The clinical, biological, histological and radiological characteristics of both groups are reported in Table 1. No major differences were found between groups for median ESSDAI at NHL diagnosis and the frequency of salivary gland hypermetabolism on PET-CT. MALT-type NHL was found in 24/25 (96%) patients including 13/13 (100%) of those MSGB+and 11/12 (92%) of those MSGB–. Six of the 13 (46%) MSGB+patients received no treatment, while all MSGB–patients were treated. Between diagnosis and 1 year of follow-up, ESSDAI scores without the NHL item did not differ (6.5 [3.5–9.5]) for the 6 untreated patients, but had significantly decreased for the 19 treated patients (3.5 [2.0–5.8]) (p=0.02).Table 1.Comparison of the pSS patients’ characteristics according to MSGB+vs. MSGB–for NHLCharacteristicMSGB+, n=13MSGB–, n=12Female/male (ratio)12/1 (12)11/1 (11)Age at NHL diagnosis, yr60 (52–72)58 (49.5–69.8)pSS duration at NHL diagnosis, yr2.(0–9)3.5 (0.8–11)ESSDAI score without NHL item9 (6–16)10.(3.5–19.8)Cryoglobulinemia+9 (69)3/9 (33)Rheumatoid factor+9/11 (82)8/8 (100)Anti-SSA antibody+10 (77)7 (58)Anemia (Hb<12g/dL)2 (15)6/11 (55)Gammaglobulins, g/L12.4 (10.8–16.9)16.1 (12.3–20.0)Histology MALT-type lymphoma13 (100)11 (92) Diffuse-large B-cell lymphoma0 (0)1 (8)[18F]FDG-PET–CT, SUV max >4.7 Parotid or submandibular gland6/12 (50)3/10 (30) Lymph nodes1/12 (8)3/10 (30)Results are presented as number (%) or median (IQR).Conclusion:Our results showed that, when MALT lymphoma is suspected, MSGB contributed to diagnosing NHL, either at initial assessment or during pSS evolution, enabling MALT lymphoma diagnosis in at least a third of NHL patients and >50% when MSGB was obtained systematically. Thus, MSGB might avoid the need for a more invasive procedure. Moreover, our findings suggest MSGB should be obtained at pSS diagnosis, and repeatedly during follow-up, when NHL, especially MALT, is suspected.Disclosure of Interests:Simon Parreau: None declared, Raphaèle Seror Consultant of: BMS UCB Pfizer Roche, Benjamin Terrier: None declared, Barbara Burroni: None declared, Céline Jamart: None declared, Alexis Régent: None declared, Gaetane Nocturne: None declared, Luc Mouthon: None declared, Xavier Mariette Consultant of: BMS, Gilead, Medimmune, Novartis, Pfizer, Servier, UCB, Véronique LE GUERN Grant/research support from: UCB for GR2 study (to our institution)
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