Outcomes of Patients With Acute Vogt–koyanagi–harada Disease Treated With Intravenous Corticosteroid Pulse Followed by the Slow Tapering of Oral Corticosteroid Therapy

Purpose We investigated the treatment outcomes of patients with acute Vogt–Koyanagi–Harada (VKH) disease and assessed the differences between patients with no inflammation worsening and those with persistent or worsening inflammation. Potential factors responsible for eyes with low visual outcomes were also investigated.Methods We retrospectively reviewed the clinical records of patients with acute VKH disease who first visited us between 2009 and 2018 and were followed up for >300 days. Clinical characteristics, treatments, and posttreatment conditions were assessed. Patients were classified into no inflammation worsening (acute–resolved [AR]) and inflammation worsening (chronic–recurrent [CR]) groups based on conditions after 6 months from disease onset.Results This study assessed 62 eyes from 31 patients (mean age: 52.8 years). One patient was treated with topical treatment alone and showed poor visual outcomes. In total, 30 patients were treated with methylprednisolone pulse followed by the slow tapering of oral prednisolone; 73% of them developed AR and 27% CR. Although the total prednisolone dose was higher in patients with CR disease, no significant difference was noted in the final best-corrected visual acuity (BCVA). Among the patients receiving systemic steroid, five eyes had a final BCVA of ≤0.5 due to anisometropic amblyopia, diabetic maculopathy, pre-existing macular hole, epiretinal membrane, and ellipsoid zone loss. Conclusions Patients with acute VKH disease treated with corticosteroid pulse followed by the slow tapering of prednisolone appear to demonstrate good visual outcomes, including patients with CR; most eyes with low visual outcomes have pre-existing conditions that explain low vision.

P024 Efficacy and tolerance of methotrexate in patients with Juvenile Idiopathic Arthritis

Background Juvenile idiopathic arthritis (JIA) is the most common inflammatory rheumatic disease for children. The therapeutic management depends on several factors and is based on different treatments including methotrexate (MTX). The aim of our study was to determine the efficacy and safety of MTX in JIA. Methods This is a monocentric retrospective study of 37 patients followed for JIA according to the 2001 International League of Association of Rheumatology (ILAR) criteria and treated with MTX. Socio-demographic, clinical, paraclinical and therapeutic data were collected. Disease activity was assessed by the JADAS score. Highly active JIA was defined as JADAS superior to 25. Results There were 25 boys (67.5%) and 12 girls (32.4%) with a median age of 6.3 years [4–13]. The average duration of the rheumatic disease was 2.7 years [2.5–5.3]. The type of JIA was: oligoarticular in 22 cases (59.4%), polyarticular in 10 cases (27%), arthritis related to enthesitis in 3 cases (8.1%) and systemic in 2 cases (5.4%). Twenty patients (54%) received oral corticosteroid therapy for a mean period of 1.7 years [0.6–3] with a mean daily dose of 10 mg/day of prednisone or equivalent. Oral MTX was prescribed to all patients with a mean weekly dose of 10 mg/m2 body surface [10–15]. MTX was initiated after a mean period of 6.2 months [3.1–11.4] from diagnosis. The mean treatment duration was 50 months [34–66]. Observance of MTX was 80.5%. Remission with MTX was achieved in 28 patients (75.6%) after a mean treatment duration of 7.5 months [5–11], with a mean JADAS of 5.1 [3.5–10]. Despite good observance of MTX, eight patients (21.6%) continued to have high disease activity with a mean JADAS score of 32 [25–40]. Tolerance to oral MTX was good, with side effects occurring only with 5 patients (13.5%), such as epigastralgia in 2 cases (which disappeared after switching to the intramuscular administration), a skin reaction in one case, and hepatic cytolysis reversible when stopping the treatment in 2 other cases. Conclusion MTX still has a place in the therapeutic management of JIA and appears to be a well-tolerated and effective treatment.

Hypercalcaemia és veseelégtelenség – merre induljunk?

Összefoglaló. Közleményünkben egy 63 éves férfi esetét ismertetjük, aki fáradékonyság, fogyás miatt végzett laboratóriumi vizsgálatokon igazolódó veseelégtelenség és hypercalcaemia miatt került felvételre Klinikánkra. A területen végzett röntgenvizsgálaton a koponyán frontalisan és a sacrumon csonteltérések (temporofrontalisan 13 mm-es, körülírtabb, mérsékelten intenzív árnyék és az S1-es rés sclerosisa) ábrázolódtak, ultrahangvizsgálat során lépmegnagyobbodás volt látható. Tünetei hátterében endokrin vagy malignus betegség nem igazolódott. A háttérben elsősorban myeloma multiplex merült fel, ugyanakkor azt célzott vizsgálatokkal sem megerősíteni, sem kizárni nem lehetett, így csontvelő-biopszia történt. A vesefunkció-romlás okának tisztázása végett vesebiopsziát végeztünk, melynek előzetes eredménye interstitialis nephritist véleményezett óriássejtekkel. Az angiotenzinkonvertáló enzim szérumszintjének ez okból történő vizsgálata emelkedett szintet mutatott, így esetünket Boeck-sarcoidosis extrapulmonalis manifesztációjának tartottuk. Per os szteroidkezelésre a beteg tünetei egyértelmű regressziót mutattak. A csontvelő- és vesebiopszia eredménye megerősítette a Boeck-sarcoidosis diagnózisát. A sarcoidosis ezen extrapulmonalis formája hypercalcaemiával és veseérintettséggel – de tüdőérintettség nélkül – rendkívül ritka, különös tekintettel a vesét érintő formára. Hypercalcaemia nagyjából 7,9%-ban, veseelégtelenség 1,4%-ban fordul elő. Ezen eset alapján fontos hangsúlyozni, hogy a hypercalcaemia és a veseelégtelenség hátterében a gyakoribb endokrin, malignus, hematológiai okok mellett a Boeck-sarcoidosisnak is fel kell merülnie a differenciáldiagnosztika során. Orv Hetil. 2021; 162(13): 514–518. Summary. We present the case of a 63-year-old male patient who was admitted to our Clinic with fatigue, weight loss, hypercalcemia, renal insufficiency and anemia. X-ray showed lesions on the frontal skull and sacral region. On abdominal ultrasound, splenomegaly was detected. Based on these, myeloma multiplex was the most likely initial diagnosis; this, however, could not be confirmed with targeted serum tests, therefore bone marrow biopsy was performed. To clarify the underlying cause of decreased kidney function, renal biopsy was performed, the preliminary results of which revealed interstitial nephritis accompanied by giant cells. Serum angiotensin converting enzyme level was elevated, which led to the diagnosis of Boeck sarcoidosis with extrapulmonary manifestations. Oral corticosteroid therapy was commenced that was followed by regression of the patient’s symptoms and laboratory abnormalities. Both the bone marrow and the kidney biopsies supported the diagnosis of Boeck sarcoidosis. Presentation of sarcoidosis with hypercalcemia and renal insufficiency but without the involvement of the lungs is extremely rare. Hypercalcemia occurs in about 7.9% and renal insufficiency in 1.4% of the cases. Based on this case, it is important to highlight that in the background of hypercalcemia and renal failure – beside the more frequent causes such as endocrine and hematological diseases, malignancy – one is to consider the possibility of Boeck sarcoidosis as well. Orv Hetil. 2021; 162(13): 514–518.

Unilateral optic nerve relapse after acute lymphoblastic leukaemia remission

Background: The aim of this case report is to describe the clinical presentation and imaging features of a patient with optic nerve leukaemic infiltration as the first site of relapse after complete response to systemic treatment. Materials and methods: We report the case of a 23-year-old man with history of acute lymphoblastic leukaemia (ALL) in complete remission. Six months later, the ocular examination revealed decreased visual acuity. Fundus examination showed a pale optic disk with blurred margins and multiple flame-shaped and dot and blot retinal haemorrhages in his left eye. A diagnosis of leukaemic infiltration to the optic nerve was made by magnetic resonance imaging (MRI). Cytological analysis of the cerebrospinal fluid did not show any abnormal cells or blasts. Results: A course of oral corticosteroid therapy was prescribed and 20 Gy of radiation was administered to the whole brain including the left orbit. Vision was improved dramatically in the left eye. Isolated optic nerve relapse of leukaemic infiltration is of paramount importance to early diagnosis, as vision can be saved if treatment with orbital radiotherapy is initiated promptly.

Non-steroidal anti-inflammatory drugs (NSAIDs) – exacerbated respiratory disease: epidemiology, pathogenesis, clinical findings and management

Non-steroidal anti-inflammatory drugs (NSAIDs) exacerbated respiratory disease (N-ERD) are characterized by non-allergic hypersensitivity (intolerance) to NSAIDs and aspirin in patients with asthma and/or eosinophilic chronic rhinosinusitis with nasal polyps (CRSwNP). Asthma in N-ERD patients is usually characterized by eosinophilic inflammation, tends to become severe and intractable, and needs chronic oral corticosteroid therapy. For some patients recurrent CSwNP is more significant problem due to nasal congestion, anosmia, and multiple repeated surgeries. Intolerance to aspirin and other NSAIDs limits the choice of pain relievers and antipyretics. Accidental use of these medications can lead to dangerous consequences, including anaphylaxis. This review presents the current understanding of the N-ERD pathogenesis and perspective trends in therapy.

Oral Corticosteroid Relieves Post-COVID-19 Anosmia in a 35-Year-Old Patient

Coronavirus disease 2019 (COVID-19) is a highly infectious respiratory illness caused by SARS-CoV-2. Not much is yet known about this new viral disease. In our early encounter with the COVID-19, before the pandemic and during the early days of dealing with this novel viral disease in our country, we saw some cases of anosmia in patients infected by SARS-CoV-2. In many cases, this chemosensitive dysfunction persisted after the negativization of the nasopharyngeal swab. We report effective treatment of anosmia by oral corticosteroid therapy in a patient recovered from COVID-19.