Abstract Objectives To describe the clinical features, treatment, and outcome of autoimmune diseases (AD) in a cohort of patients with thymoma. Design Pathological records from three university ...hospitals, between 2005 and 2011, were reviewed to identify patients with thymoma. Patients with thymoma and AD were compared with patients with thymoma without AD. Results 47/85 (55%) cases of thymoma had AD, including myasthenia gravis (MG) (n = 33), Hashimoto's thyroiditis (n = 4), Isaac's syndrome (n = 3), Morvan syndrome (n = 2), pure red cell aplasia (n = 2), systemic lupus (n = 2), lichen planus (n = 2), and one case of each following conditions: aplastic anemia, autoimmune hemolytic anemia, Good's syndrome, pemphigus, autoimmune hepatitis, Graves' disease, limbic encephalitis, and inflammatory myopathy. Six patients (7%) presented at least 2 ADs. The median duration of follow-up after surgery was 60 months (40–78 months). In 32 patients, the diagnosis of AD preceded the diagnosis of thymoma, in 9 patients, thymoma was diagnosed at the same time as the AD and 7 patients had been operated on when they developed an AD. We found a significative difference on the Masaoka stage between the MG patients and the patients who present another AD (p = 0.028). No risk factor for developing an AD after thymectomy was identified. Conclusions We describe here the long-term follow-up of a large series of AD related to thymoma. Our results confirm previous data concerning AD occurrence in patients with thymoma and suggest that preexisting autoimmunity is not a risk factor for developing autoimmune manifestations after thymectomy.
To assess the diagnostic value of brain magnetic resonance imaging (bMRI) for the etiological diagnosis of uveitis and to establish predictive factors associated with its advantageous use.
...Retrospective study on all patients with de novo uveitis who were referred to our tertiary hospital and who underwent a bMRI between 2003 and 2018.
bMRI was contributive in 19 out of 402 cases (5%), among patients with a contributive bMRI, 68% had neurological signs. Univariate analysis established that neurological signs (p < .001), granulomatous uveitis (p = .003), retinal vasculitis (p = .002), and intermediate uveitis (p < .001) were all significantly associated with a contributive bMRI. Multivariate analysis confirms the significant association of neurological signs (p < .001) and intermediate uveitis (p = .01).
bMRI appears to be a relevant exam in specific cases; intermediate/posterior uveitis or panuveitis accompanied by neurological signs, retinal vasculitis, or in patients older than 40, to rule out an oculocerebral lymphoma.
ACE: Angiotensin-Converting Enzyme; bMRI: Magnetic Resonance Imaging; CBC: Complete Blood cell Count; BMRI: Brain Magnetic Resonance Imaging; CT: Computerized Tomography; MS: Multiple Sclerosis; NS: Neurological Signs; OCL: Oculocerebral Lymphoma; RIS: Radiologically Isolated Syndrome
To assess the clinical features and outcome of lymphoma when associated with sarcoidosis and to determine whether this association gives lymphoma a better prognosis.
Multicentre retrospective cohort ...study.
Retrospective chart review.
Twenty-one patients were included (9 males, 12 females). Median age at sarcoidosis diagnosis was 48 years (range: 24-68 years). In 14 cases, lymphoma occurred within a previously known sarcoidosis. Five patients received a concomitant diagnosis of sarcoidosis and lymphoma, whereas lymphoma preceded sarcoidosis in two patients. Three patients were diagnosed with Hodgkin's lymphoma and 18 patients with non-Hodgkin's lymphoma (diffuse large B-cell lymphoma (DLBCL) (n = 11), follicular lymphoma (n = 2), chronic lymphocytic leukemia/small lymphocytic lymphoma (n = 2), anaplastic large cell lymphoma ALK + (n = 1), angioimmunoblastic T-cell lymphoma (n = 1) and T-cell prolymphocytic leukemia (n = 1)). Thirteen patients were alive and in complete remission. Median age at the time of diagnosis of sarcoidosis was lower in patients with concomitant lymphoma compared with patients with sarcoidosis preceding lymphoma (34 years vs. 51 years, P = 0.01). Patients presenting with DLBCL associated with sarcoidosis were compared with DLBCL without sarcoidosis. No statistical difference was found in the risk of death or progression between the two groups (P = 0.685).
We report here the largest series of lymphoma associated sarcoidosis patients. As opposed to previous studies, we observed a predominance of patients with DLBCL. Our study confirms the concept of the sarcoidosis-lymphoma syndrome. Large B-cell lymphoma does not have a better prognosis when associated with sarcoidosis.
Background
Transverse myelitis is a rare complication of systemic lupus erythematosus (SLE). This retrospective multicentre study identifies the prognostic factors in a relatively large patient ...series.
Patients and methods
Twenty patients fulfilled the SLE criteria of the ACR classification and the Transverse Myelitis Consortium Working Group. A severe neurological flare was defined as muscle strength grade <3/5 in more than half the muscle groups at the motor neurological level. Inability to run or another significant ambulation-unrelated disability was considered as ‘unfavourable neurological outcome’.
Results
Myelitis was the first SLE symptom in 12 patients; in the eight others, it occurred 8.6 years (median delay) after SLE onset. Eleven patients presented severe neurological impairments. The treatment included corticosteroids in all patients associated with intravenous cyclophosphamide in 11 and/or hydroxychloroquine in 14. Unfavourable outcomes were observed in 53% of the patients at six months and in 28% at end of follow-up (median: 5.9 years). An initial severe neurological impairment and no cyclophosphamide use were associated with unfavourable neurological outcomes at six months and at end of follow-up, respectively.
Conclusion
Transverse myelitis may reveal SLE or occur more than 10 years after SLE diagnosis. The initial severity of the neurological flare (with paraplegia) is the main prognostic marker. The study provides arguments for cyclophosphamide use.
Abstract
Background
Renal and splenic infarctions are close entities, with few data concerning their clinical, biological and radiological features.
Aim
The aim of this study was to compare the ...clinical presentations, etiologies and outcomes of acute renal infarctions (RI) and splenic infarctions (SI).
Design
A retrospective multicentric cohort study included patients of the 6 university hospitals in Lyon with RI, SI, or associated RI-SI infarctions was conducted.
Methods
All consecutive cases diagnosed by CT imaging, between January 2013 and October 2016, were included. The exclusion criteria were causes of infarction that did not require additional investigations.
Results
A total of 161 patients were selected for analysis: 34 patients with RI, 104 patients with SI and 23 patients with both RI-SI. Mean ± SD age of patients was 63.2 ± 16.6 years; 59.6% were male. Only 5/161 (3.1%) were healthy prior to the event. The main symptoms were diffuse abdominal pain (26.4%), followed by nausea/vomiting (18.3%) and fever (16.4%).The causes of RI or SI varied significantly within the three groups. Hypercoagulable state was associated with SI, and embolic disease and arterial injury were associated with RI. Extensive (i.e.>2/3 of organ volume) (OR 6.22, 95%CI 2.0119.22) and bilateral infarctions (OR 15.05, 95%CI 1.79–126.78) were significantly associated with hemodynamic shocks. The survival at 1 month follow-up did not significantly differ between the three groups.
Conclusion
Acute RI and SI are heterogenous entities in regards to their clinical presentation, etiology, associated venous or arterial thrombosis, but prognoses were not different at short term follow-up.
Sarcoidosis is one of the leading causes of inflammatory eye disease. Any part of the eye and its adnexal tissues can be involved. Uveitis and optic neuropathy are the main manifestations, which the ...internists face. This review reports the state of knowledge for these two ocular involvements and proposes an assessment-algorithm for sarcoidosis in patients with suspected sarcoid uveitis. Two groups of patients with sarcoid uveitis can be distinguished: one young and multiethnic group in which ophthalmological findings are various and another group of elderly Caucasian women with mostly chronic posterior uveitis. Clinically isolated uveitis revealing sarcoidosis remains a strictly ocular condition in a large majority of cases. Although it could be a serious condition involving functional prognosis, an early recognition in addition to a growing therapeutic arsenal including intravitreal implant seems to have improved visual prognosis of the disease in the last years. Systemic corticosteroids are indicated when uveitis does not respond to topical corticosteroids or when there is bilateral posterior involvement, especially macular edema and occlusive vasculitis. In up to 25% of cases that require an unacceptable dosage of corticosteroids to maintain remission, additional immunosuppression is used, including methotrexate, azathioprine, and mycophenolate mofetil. Regarding systemic sarcoidosis, infliximab and adalimumab have been successfully used for the treatment of refractory or sight-threatening disease. Optic neuropathy often affects women of African and Caribbean origin. Some authors recommend that patients be treated with high-dose corticosteroids and concurrent immunosuppression from the onset for this manifestation, which may be associated with a poorer outcome.
Background: Spinal cord involvement in sarcoidosis is rare, occurring in <1% of patients with sarcoidosis.
Methods: We report seven cases of spinal cord sarcoidosis, seen in two French hospitals ...over a 13‐year period. Presentation of disease, methods of diagnosis and response to treatment, with quantification according to the reduction of the modified Rankin scale (MRS), were noted.
Results: Six patients presented insidious paresthesias or weakness and one a sudden paraplegia. Average MRS at diagnosis was to 2. Spine MRI showed one or several intramedullary lesions in all cases. Diagnosis was confirmed by extra‐neural tissue biopsies in all cases, including mediastinoscopy (two patients), coelioscopy (one patient), bronchoscopy (one patient), salivary gland biopsy (one patient) and skin biopsy (two patients). The average follow‐up for the group was 49.4 months. All patients responded to corticosteroid therapy with a median reduction of MRS of one point. Five patients received immunosuppressive therapy: cyclophosphamide (two patients), methotrexate (two patients), azathioprine (one patient), mycophenolate mofetyl (one patient), with an inconstant benefit. Patients who received cyclophosphamide presented severe fungaemia.
Conclusion: Based on our study and literature analysis, we propose an algorithm for treatment of spinal cord sarcoidosis.
Recurrent FUO (fever of unknown origin) is a rare subtype of FUO for which diagnostic procedures are ill-defined and outcome data are lacking.
We performed a retrospective multicentre study of ...patients with recurrent FUO between 1995 and 2018. By multivariate analysis, we identified epidemiological, clinical and prognostic variables independently associated with final diagnosis and mortality.
Of 170 patients, 74 (44%) had a final diagnosis. Being ≥ 65 years of age (OR = 5.2; p < 0.001), contributory history (OR = 10.4; p < 0.001), and abnormal clinical examination (OR = 4.0; p = 0.015) independently increased the likelihood of reaching a diagnosis, whereas lymph node and/or spleen enlargement decreased it (OR = 0.2; p = 0.004). The overall prognosis was good; 58% of patients recovered (70% of those with a diagnosis). Twelve (7%) patients died; patients without a diagnosis had a fatality rate of 2%. Being ≥ 65 years of age (OR = 41.3; p < 0.001) and presence of skin signs (OR = 9.5; p = 0.005) significantly increased the risk of death.
This study extends the known yield of recurrent FUO and highlights the importance of repeated complete clinical examinations to discover potential diagnostic clues during follow-up. Moreover, their overall prognosis is excellent.
To describe the main characteristics and the treatment of cryptococcosis in patients with sarcoidosis.
Multicenter study including all patients notified at the French National Reference Center for ...Invasive Mycoses and Antifungals.
Retrospective chart review. Each case was compared with two controls without opportunistic infections.
Eighteen cases of cryptococcosis complicating sarcoidosis were analyzed (13 men and 5 women). With 2749 cases of cryptococcosis registered in France during the inclusion period of this study, sarcoidosis accounted for 0.6% of all the cryptococcosis patients and for 2.9% of the cryptococcosis HIV-seronegative patients. Cryptococcosis and sarcoidosis were diagnosed concomitantly in four cases; while sarcoidosis was previously known in 14/18 patients, including 12 patients (67%) treated with steroids. The median rate of CD4 T cells was 145 per mm(3) (range: 55-1300) and not related to steroid treatment. Thirteen patients had cryptococcal meningitis (72%), three osteoarticular (17%) and four disseminated infections (22%). Sixteen patients (89%) presented a complete response to antifungal therapy. After a mean follow-up of 6 years, no death was attributable to cryptococcosis. Extra-thoracic sarcoidosis and steroids were independent risk factors of cryptococcosis in a logistic regression model adjusted with the sex of the patients.
Cryptococcosis is a significant opportunistic infection during extra-thoracic sarcoidosis, which occurs in one-third of the cases in patients without any treatment; it is not associated to severe CD4 lymphocytopenia and has a good prognosis.
Adult-onset Still's disease is a rare and difficult to diagnose multisystemic disorder considered as a multigenic autoinflammatory syndrome. Its immunopathogenesis seems to be at the crossroads ...between inflammasomopathies and hemophagocytic lymphohistiocytosis, the most severe manifestation of the disease. According to recent insights in the pathophysiology and thanks to cohort studies and therapeutic trials, two phenotypes of adult-onset Still's disease may be distinguished: a systemic pattern, initially highly symptomatic and with a higher risk to exhibit life-threatening complications such as reactive hemophagocytic lymphohistiocytosis, where interleukin-1 blockade seems to be very effective, a chronic articular pattern, more indolent with arthritis in the foreground and less severe systemic manifestations, which would threat functional outcome and where interleukin-6 blockade seems to be more effective. This review focuses on these data.
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