Mediterranean Journal of Rheumatology最新文献

Objective: Kawasaki disease (KD) and atypical cat-scratch disease (CSD) can manifest with fever and similar non-specific symptoms.

Methods: We report the case of an immunocompetent child who presented with signs and symptoms consistent with KD, which overlapped with those of atypical CSD (hepatosplenic form). Subsequently, we conducted a literature review to identify paediatric cases of KD and CSD.

Results: We present the case of a 6-year-old girl with fever, abdominal pain, non-purulent bilateral conjunctivitis, maculopapular rash on the trunk, bilateral cervical lymphadenopathy, and oral mucosa erythema, meeting KD diagnostic criteria. Echocardiography revealed coronary artery dilatations. Despite initial intravenous immunoglobulin (IVIG) treatment eighteen hours later the general condition worsened, with the recurrence of fever, diffuse myalgia, severe abdominal pain, and vomiting. A detailed history revealed a cat scratch three weeks before onset, along with an erythematous nodule on the thumb, axillary lymphadenopathy, a typical hypoechoic splenic lesion in abdominal ultrasonography, and highly elevated IgM and IgG antibodies for Bartonella henselae titres, leading to a diagnosis of atypical CSD. Successful treatment involved a three-month course with erythromycin. Our literature review revealed five cases of co-occurring KD and CSD and six cases where CSD mimicked autoimmune diseases or malignancies.

Conclusion: The presented case illustrated the expanding spectrum of B. henselae infection and emphasised the importance of including it in the differential diagnosis of KD and prolonged fever syndromes. We suggest incorporating abdominal ultrasonography into the initial diagnostic workup, considering it to be essential before empiric therapy initiation.

We report the case of a 50-year-old female smoker presenting with isolated algodystrophy of the navicular bone and concurrent nailfold videocapillaroscopy (NVC) abnormalities. This observation raises the possibility of an association between microvascular alterations and the etiopathogenesis of algodystrophy. NVC revealed enlarged loops, tortuosity, pericapillary oedema, and microhaemorrhages, findings indicative of impaired microcirculation. Magnetic resonance imaging (MRI) of the foot demonstrated bone marrow oedema consistent with algodystrophy. The patient underwent intramuscular treatment with neridronate, which resulted in full symptomatic remission and radiological resolution at three months. This case highlights the potential role of microvascular dysfunction in algodystrophy and suggests that NVC may serve as a valuable diagnostic tool in patients with unexplained regional pain syndromes. Future studies are required to establish a definitive causal relationship.

Background: Managing pregnancy and other reproductive concerns in individuals with rheumatic disease depends on the type of disease, its activity, co-morbidities, and other factors. This study's primary objective was to evaluate rheumatologists' knowledge, attitudes, and practices in this field.

Methods: This cross-sectional web-based online survey was conducted from October to November 2022 among Indian Rheumatologists. Information was sought regarding knowledge and attitudes in managing pregnancy and reproductive health-related issues, their approach, barriers faced, patient perspectives, and possible solutions.

Results: In total, 122 rheumatologists participated in the survey. Most rheumatologists were familiar with the British Society of Rheumatology (BSR) 2023 and American College of Rheumatology (ACR) 2020 guidelines on prescribing drugs in pregnancy and breastfeeding. Contraception and drug compatibility with breastfeeding were answered appropriately by 80% of the participants. Most (77.9%) were moderately to extremely confident in managing pregnant patients with rheumatic diseases. However, the majority were only slightly or not at all confident in discussing oocyte preservation(63%) and assisted reproductive techniques(65.9%). Almost all reported that their patients had fears about planning a pregnancy due to the disease (92%), and they frequently came across patients prematurely stopping their medicines after pregnancy was confirmed (50.8%).The majority of the respondents (91.7%) felt lack of proper treatment and regular follow-ups were the main contributing factors to poor pregnancy outcomes.

Conclusion: Rheumatologists treating patients with reproductive issues in rheumatic diseases have certain knowledge gaps; filling these gaps can boost their confidence in managing these patients. There is scope for improving collaboration with obstetricians, and patient education.

Objective: This report presents a case of methotrexate (MTX)-associated hypersensitivity pneumonitis (HP) after 15 years of use and reviews the literature on its diagnosis, treatment, symptoms, and risk factors.

Case: A 65-year-old female patient with rheumatoid arthritis (RA) presented with shortness of breath and a non-productive cough after 15 years of stable MTX treatment. Chest X-ray revealed bilateral ill-defined infiltrates, and high-resolution computed tomography (HRCT) showed diffuse ground-glass opacities. All diagnostic tests for infection were negative. Suspecting MTX-associated HP, MTX was discontinued, leading to significant clinical improvement.

Discussion: HP is the most common form of pulmonary toxicity associated with MTX. Symptoms typically include dry cough and dyspnoea in 80% of patients. Significant eosinophilia may be observed. Risk factors include age over 60, diabetes, pre-existing lung disease, hypoalbuminemia, RA-related lung involvement, renal dysfunction, male gender, and Daily dose. The diagnosis of MTX-associated HP is a diagnosis of exclusion. Differential diagnosis can be challenging, as it may overlap with other conditions. Although diagnostic criteria have been reported, diagnosis is primarily based on clinical, radiological, and laboratory findings, along with treatment response. Management involves discontinuation of MTX and corticosteroid therapy. While MTX-associated HP generally follows a favourable course with most patients achieving full recovery, reported mortality rates can be as high as 17.6%.

Conclusion: While MTX-associated HP is usually reported within the first years of treatment, it can also occur after prolonged use. Clinicians should consider this possibility in the differential diagnosis, as early detection can result in treatable outcomes.

Background: Lupus enteritis (LE) is the most common serious gastrointestinal manifestation of SLE. Indian literature on LE is limited, while most of the data comes from China and Western series with recent efforts on developing prediction models for its occurrence & recurrence.

Objectives: The objectives of the study were to analyse clinical and laboratory parameters of LE, to compare recurrent versus non-recurrent LE and to identify predictors of its recurrence and mortality.

Method: Patients with LE who were admitted to the Rheumatology ward from 2018-2022 were considered cases. For cases, symptoms, abdominal CT findings, and outcome (recurrence or death) were recorded. Logistic regression was used for the prediction of recurrence and mortality.

Result: Among 48 LE patients, 45 were female. The mean (± SD) age of the first enteritis episode was 27.04± 8.92 years. The most frequent extra-gastrointestinal manifestations were nephritis and arthritis (54% of cases). Mean disease duration, lupus nephritis, arthritis, and mean SLEDAI were higher in non-LE patients. Thirteen (27.1%) patients had recurrent LE & hydroureteronephrosis was more prevalent in them (p = 0.002). The logistic regression model using dsDNA complements and albumin failed to predict recurrence. Multiple repetitions of the confusion matrix for the mortality prediction model yielded variable ρ scores, suggesting insignificant accuracy prediction.

Conclusion: Colon and bladder involvement is more frequent in recurrent LE. Anti-dsDNA antibodies, complement, disease activity index, and serum albumin failed to predict recurrence and mortality in our cohort.

Objectives: To examine the performance of the minor salivary gland biopsy (MSGB) to diagnose Sjögren's syndrome (SS) and to identify predictive factors for MSGB's positivity in Tunisian SS-suspected patients.

Methods: In a retrospective study, histopathological evaluation of MSGB from SS suspected- patients were examined. The classifications of the American-European Consensus Group (AECG, 2002) and the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR, 2016) have been applied. We classified a positive MSGB when a focus score ≥ 1 and/or Chisholm and Mason grading ≥ 3 were observed. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of MSGB were assessed, and the area under the ROC curve was performed to evaluate its diagnostic accuracy.

Results: One hundred and two MSGBs were examined. They were positive in 48 patients (47%). For the positive diagnosis of SS, MSGB had 77,6% sensitivity, 93,2% specificity, 93,8% PPV and 75,9% NPV. With an air under the curve (AUC) of 0.854, MSGB was considered an excellent discriminating test in SS diagnosis. Keratoconjunctivitis sicca (p=0.04), elevated erythrocyte sedimentation rate (p=0.036), leukopenia (p=0.025), positive antibodies: anti-Ro/SSA (p=0.029), anti-Ro/SSA, anti-La/SSB (p=0.037), antinuclear (p=0.01), anti-extractable nuclear antigen (p=0.04), positive rheumatoid factor (p=0.032), positive elevated IgG levels (p=0.03) and abnormal unstimulated whole salivary flow rate (p=0.002) were predictive of a positive MSGB.

Conclusion: In cases of suspected SS, a predictive scoring system incorporating these clinical and biological factors will streamline MSGB indications and serve as a diagnostic tool for positive SS diagnosis in research studies.

Autoinflammatory diseases affecting the NLRP3 gene are rare autosomal dominant disorders presenting with episodic organ limited and systemic inflammation. We report three patients with cryopyrinopathies. Our first case is a 4-year-old boy with a history of periodic fever, failure to thrive, and raised intracranial pressure. The second case is a 6-year-old boy with similar complaints, also with bilateral uveitis. The third is a 24-year-old gentleman with periodic fever and early hearing loss, also with a novel presentation of sacroiliitis. Our case series demonstrates that there should be a low clinical threshold indicating genetic testing in any child who displays features of autoinflammation in combination with an urticarial rash, musculoskeletal manifestations, hearing loss, and chronic aseptic meningitis with macrocephaly. Furthermore, despite anakinra being a cornerstone in treating NLRP-3 AID, there is an unmet clinical need to provide access to alternatives such as colchicine and thalidomide in resource-limited settings.

Background: Secukinumab is a monoclonal antibody targeting interleukin-17A (IL-17A), approved for the treatment of moderate-to-severe plaque psoriasis and psoriatic arthritis. While effective for many patients, it has been associated with paradoxical reactions such as exacerbation of psoriasis or drug-induced dermatitis. This case report presents a unique occurrence of severe palmoplantar psoriasis triggered by Secukinumab in a patient with psoriatic arthritis and multiple sclerosis (MS), necessitating a switch to another biologic therapy.

Case presentation: A 41-year-old female with a complex medical history including MS presented with severe desquamation and erythematous skin lesions on the hands, feet, and legs following treatment with secukinumab for psoriatic arthritis that necessitated discontinuation of secukinumab. A biopsy suggested drug eruption, with a differential diagnosis including palmoplantar pustular psoriasis. After multidisciplinary consultation, treatment was switched to guselkumab, an IL-23 inhibitor, which resulted in clinical improvement.

Conclusion: This case highlights the potential for paradoxical reactions to IL-17 inhibitors and the efficacy of IL-23 inhibitors as an alternative therapy. Multidisciplinary approach was crucial for optimal patient management.