Modern rheumatology case reports最新文献

Diffuse alveolar haemorrhage (DAH) is an uncommon and potentially life-threatening occurrence in systemic lupus erythematosus, involving bleeding into the alveolar space caused by the disruption of the alveolar capillary basement membrane. We present a 24-year-old Persian woman with a complaint of progressively worsening shortness of breath following the administration of intramuscular Rhogam after 3 days. According to her worsening clinical condition, the pregnancy was terminated. She was admitted to the intensive care unit and intubated. Simultaneously, she developed fungal and bacterial pneumonia tolerant to therapies. After several investigations, the patient was finally diagnosed with systemic lupus erythematosus along with DAH, as the first presentation of the disease. This is the first case of a pregnant woman experiencing DAH as a lupus flare following Rhogam injection. Clinicians should be aware of the mimicry nature of lupus and the importance of immune reactions in these patients.

Behçet's disease (BD) is a chronic, relapsing, systemic vasculitis of unknown aetiology that affects blood vessels of all sizes, potentially leading to severe complications such as coronary artery aneurysms. This report describes the case of a 33-year-old woman with BD who presented with recurrent chest pain. Imaging revealed a large saccular aneurysm in the left anterior descending artery. Management involved multiple percutaneous coronary interventions to stabilise the aneurysm, alongside infliximab, a tumour necrosis factor-alpha inhibitor, to control the underlying vasculitis. The patient has remained in clinical remission for over 3 years, providing additional evidence supporting the role of infliximab in addressing vascular complications in BD. This case highlights the challenges in managing coronary artery aneurysms in BD and emphasises the need for further research into the long-term safety and efficacy of infliximab for such cases.

IgG4-related disease (IgG4-RD) is a systemic, immune-mediated, fibroinflammatory disorder that affects multiple organs. Histopathologically, the supportive findings of IgG4-RD include dense lymphocytic infiltrates, obliterative phlebitis, storiform fibrosis, and elevated numbers of IgG4-positive plasma cells. However, the presence of granulomatous inflammation is generally considered highly atypical, suggesting alternative diagnoses such as sarcoidosis and lymphoma. Here, we present a case of IgG4-RD involving granulomatous lymphadenopathy. Labial salivary gland biopsy findings were consistent with IgG4-related sialadenitis. Elevated serum IgG4 levels, hypocomplementemia, and abnormal imaging findings in the kidneys and pancreas indicated an association with IgG4-RD. The patient was treated with prednisolone, which resulted in a significant improvement in the serum IgG4 and complement levels and a notable reduction in lymph node swelling. Although granulomatous inflammation is rare, integrating clinical, serological, radiological, and pathological parameters can ensure an accurate assessment within the appropriate clinicopathological context.

A 31-year-old woman visited our hospital with swelling and pain in both forearms of 2 months' duration, followed by swelling and pain in both thighs. Her medical history included bronchial asthma at the age of 18 years. After the birth of her first child at 30 years of age, her asthma worsened and was accompanied by abdominal pain and skin rash, with no identifiable cause. Blood testing showed eosinophilia and high muscle enzyme activities, but she was anti-neutrophilic cytoplasmic antibody (ANCA)-negative. Magnetic resonance imaging of her forearms and thighs revealed strong signals on T2-weighted images in the fascia and muscle. Skin-to-muscle en bloc biopsy showed eosinophilic infiltration of muscle and small vessels. She was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA), complicated by myositis, although EGPA is usually accompanied by ANCA-positivity in approximately half of cases. Treatment was started with prednisolone alone at 0.5 mg/kg/day, and her symptoms and eosinophil count quickly improved. Clinicians should note the possibility of ANCA-negative EGPA complicated by myositis.

A paradoxical reaction refers to a response opposite to the expected effects of drug therapy. Cutaneous paradoxical reactions, which occur with the use of immunosuppressive drugs, are believed to result from treatment-induced cytokine imbalances. These reactions can manifest as various dermatological conditions, including psoriasis and palmoplantar pustulosis. Currently, no established guidelines exist for reducing or discontinuing biologics in patients with rheumatoid arthritis who experience paradoxical reactions, posing a significant challenge for clinicians managing dermatitis and the underlying diseases. This case report presents two cases of patients with rheumatoid arthritis who developed palmoplantar pustulosis as a paradoxical reaction after treatment with golimumab, a tumour necrosis factor inhibitor. Both patients achieved remission of joint and skin symptoms following treatment with peficitinib, a Janus kinase inhibitor. To the best of our knowledge, this is the first report documenting the successful treatment of paradoxical reaction-induced palmoplantar pustulosis with peficitinib. These findings suggest that peficitinib could serve as an effective alternative when tumour necrosis factor inhibitors are no longer viable. Thus, peficitinib may be a potential therapeutic option for the management of rheumatoid arthritis patients with palmoplantar pustulosis.

Tocilizumab (TCZ) is effective for inducing remission in adult-onset Still's disease (AOSD), but its use may occasionally trigger macrophage activation syndrome (MAS). The rationale for re-introducing TCZ in patients with a history of MAS is not well established. Here, we report a case of successful re-administration of TCZ for an AOSD relapse in a patient with a prior history of MAS during TCZ therapy. A 67-year-old woman, initially treated with TCZ for polyarthritis, developed MAS associated with AOSD. MAS was resolved with glucocorticoid pulse therapy, high-dose glucocorticoids, and cyclosporine A. However, AOSD relapsed during glucocorticoid tapering. Methotrexate, cyclosporine A, and repeated glucocorticoid pulses failed to control the disease. Following another glucocorticoid pulse, TCZ (8 mg/kg weekly) was re-introduced intravenously. This approach allowed successful glucocorticoid tapering and long-term remission. This case highlights the complexities of managing AOSD: while the initial TCZ therapy may have contributed to the onset of MAS, the subsequent re-introduction of TCZ enabled effective disease control and sustained remission.

Patients with rheumatoid arthritis (RA) receiving immunosuppressive therapy including methotrexate (MTX) are at risk of developing lymphoproliferative disorder (LPD). Herein, we report the case of a 61-year-old man who has been treated with MTX and sulfasalazine for seropositive RA since the age of 52 years. He underwent diffusion-weighted whole-body imaging with background signal suppression (DWIBS), which revealed high-intensity lesions in the affected lymph nodes of the cervical, clavicular, and axillary regions. Follow-up DWIBS after MTX withdrawal showed the suppression or disappearance of the high-intensity lesions. This case demonstrates the potential of DWIBS as a new standard imaging modality for MTX-LPD in patients with RA in clinical practice.

Skin ulcers sometimes appear in patients with antimelanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) and are usually associated with disease activity. Here, we report a case of a 41-year-old woman with anti-MDA5 antibody-positive DM, who developed refractory skin ulcers during the remission induction therapy, which were proven to be associated with clinically silent Staphylococcus aureus bacteraemia with septic thrombi in her lung. The patient was referred to our hospital for the treatment of amyopathic DM with interstitial lung disease. Anti-MDA5-positive DM was diagnosed, and she was treated with triple therapy combined with tofacitinib because poor prognostic factors existed. Although the remission induction therapy improved most of the symptoms, she developed erythematous rashes with ulcers on her left auricle and forearm, which were refractory to topical immunosuppressive medications. Despite the absence of systemic symptoms or elevated inflammatory markers, blood and wound cultures revealed S. aureus, and a new cavitary lesion was detected in her left lung. Subsequent antibiotic therapy resolved both the cutaneous and pulmonary lesions. This case highlights the importance of considering bacteraemia and performing blood cultures when DM-related skin ulcers resist conventional treatments, even without fever during immunosuppressive therapy.

The recombinant zoster vaccine (RZV) is immunologically and clinically effective in immunosuppressed patients. Though rheumatoid arthritis (RA) and the Janus kinase inhibitor (JAKi) increase the risk of herpes zoster (HZ) infection, breakthrough cases in which a HZ infection followed RZV administration are rare. We report herein a 63-year-old female patient with seropositive RA who experienced a HZ infection despite receiving the RZV. She had been receiving tocilizumab, methotrexate, and low-dose prednisolone until tocilizumab was switched to upadacitinib 4 weeks after two RZV administrations, which resulted in 63 weeks' remission. Her current admission was for a painful rash consisting of blisters and erythema on the right nasal alar and lips corresponding to the right V2 segment of the trigeminal nerve. HZ was diagnosed and treated for 7 days with intravenous acyclovir, which alleviated the symptoms. JAKi can suppress a range of immunogenic mechanisms which underlie the efficacy of the RZV. The present patient was expected to respond favourably to the RZV because JAKi had not been administered prior to the vaccinations; however, the later start of JAKi therapy caused a breakthrough HZ infection. Immunocompromised patients have a higher risk of severe HZ, including the disseminated form, but breakthrough cases are relatively rare. The RZV is recommended as prophylaxis against HZ as well as means of mitigating its severity when it does occur.

Polymyalgia rheumatica (PMR) is a common inflammatory disorder characterized by myalgia/stiffness in proximal hip and shoulder girdle, elevated C reactive protein, and erythrocyte sedimentation rate, but its pathogenesis is not fully elucidated. We report three cases of PMR who do not respond adequately to standard treatment. Those patients had typical symptoms of myalgia and muscle weakness, with elevated C reactive protein in absence of creatine kinase elevation. Muscle specimen showed the findings of vasculitis in all cases; therefore, muscular-limited vasculitis may be an underlying pathology in PMR in those refractory cases.