Modern rheumatology case reports最新文献

A 55-year-old woman presented to our hospital with arthritis of the hands. Tests for anti-cyclic citrullinated peptide antibodies and rheumatic factor were negative, and seronegative rheumatoid arthritis was diagnosed. The patient was treated with methotrexate and certolizumab (tumour-necrosis factor-α inhibitor). Disease activity was controlled with the drugs, but after 14 months, erythematous plaques with scaling appeared on the palms and feet. Skin biopsy revealed bullous lesions with aseptic abscess formation, leading to the diagnosis of palmoplantar pustulosis and pustulotic arthro-osteitis. The patient had a family history of palmoplantar pustulosis. The skin lesions improved after discontinuation of certolizumab and treatment with ointments and phototherapy. This case suggests that tumour-necrosis factor-α inhibitors may potentially trigger palmoplantar pustulosis in patients with a family history (genetic factor) of the condition.

Patellar tendon rupture is a severe complication following total knee arthroplasty (TKA). We encountered a case of rheumatoid arthritis with an incomplete rupture of the patellar tendon post-TKA. An 84-year-old woman was diagnosed with an incomplete rupture of the right patellar tendon 3 months post-TKA of her right knee. The patient exhibited a 45° extension lag 6 months post-TKA, necessitating reconstruction surgery. Intraoperative findings revealed incomplete rupture of the patellar tendon that was stretched out, diagnosed as incomplete patellar tendon rupture. Due to knee valgus instability (passive knee valgus showed 20°), the thickness of the tibial insert was adjusted from 11 to 15 mm, resulting in improved valgus instability. The scarring region of the patellar tendon was resected to 10 mm in length, and the tendon was repaired using an ultra-high molecular weight polyethylene cable (Nesplon cable) and Krackow suture. The repair was secured by making an 8-figure pattern with the cable. After the reconstruction surgery, the knee was immobilised at 0° extension for 3 weeks, followed by the initiation of range-of-motion exercises. Three months later, the extension lag was reduced to -15°, and the patient could walk without orthosis and reported neither instability nor surgical site infection at 8 months after the surgery. In conclusion, this case is notable due to the rarity of incomplete (stretched out) patellar tendon rupture post-TKA and demonstrates the effectiveness of Nesplon cable with Krackow suture in reconstruction surgery.

Most reported cases of large vessel vasculitis (LVV) following coronavirus disease 2019 (COVID-19) have involved adults, with paediatric cases being rare. We present the case of a 14-year-old boy who developed LVV following COVID-19. Initially, he presented with fever and cough, and nasopharyngeal polymerase chain reaction testing confirmed COVID-19. His symptoms spontaneously resolved without specific COVID-19 treatments. However, 10 days after contracting COVID-19, his fever recurred and his inflammatory markers were significantly elevated. His condition did not meet the criteria for Kawasaki disease or multisystem inflammatory syndrome in children associated with COVID-19. Contrast-enhanced computed tomography revealed arterial wall thickening in the aorta and carotid arteries, indicative of LVV. Upon initiation of high-dose immunoglobulin therapy and aspirin, his fever subsided and his inflammatory markers and imaging findings normalised. Differential diagnosis ruled out infections, immune disorders, and Takayasu arteritis (TAK), a common cause of aortitis in children. Over a 1-year follow-up period, there were no recurrence and no stenotic lesions in large vessels. This finding suggests that the patient experienced transient LVV following COVID-19. Cytokine profile analysis performed before and after treatment revealed elevated levels of interleukin (IL)-6, IL-8, and IL-12/IL-23p40, typically associated with the active phase of TAK. Importantly, IL-17A and tumour necrosis factor-α levels were normal, as elevations in these cytokines have been linked to TAK recurrence. Notably, some cases of LVV following COVID-19 do not respond well to treatment; further research, including case accumulation and cytokine profile analysis, is needed to better predict prognosis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis preceded by bronchial asthma or allergic sinusitis and accompanied by peripheral blood eosinophilia. Immunosuppressive drugs, such as cyclophosphamide in addition to high-dose glucocorticoids (GCs), are recommended for induction of remission in patients with severe EGPA. Although mepolizumab is widely recognised as remission induction therapy in nonfatal/nonorgan disabling or relapsed/refractory EGPA, its efficacy and safety in induction of remission for severe cases have been ambiguous. In this context, we report a case of myeloperoxidase antineutrophil cytoplasmic antibody-positive severe EGPA in which the patient had a favourable course using mepolizumab as an induction remission therapy. The patient, a 74-year-old man, had myeloperoxidase antineutrophil cytoplasmic antibody-positive severe EGPA with alveolar haemorrhage. High-dose GCs and intravenous cyclophosphamide were started as remission induction therapy. However, after the initiation of intravenous cyclophosphamide, alveolar haemorrhage worsened, and there was development of opportunistic infections, such as aspergillus and cytomegalovirus antigenaemia. Treatment with the antifungal drug voriconazole and the antiviral drug ganciclovir was started for opportunistic infection, and the treatment for EGPA was switched from intravenous cyclophosphamide to mepolizumab. As a result, alveolar haemorrhage improved, GCs were reduced, and the infection also improved. Mepolizumab as remission induction therapy for severe EGPA were thought to be appropriate and effective treatment in this case. However, the efficacy and safety of mepolizumab for this purpose require comprehensive evaluation.

Eosinophilic fasciitis involves collagenous thickening of the subcutaneous fascia, hypergammaglobulinaemia, and peripheral eosinophilia, manifesting as erythema and oedema of the extremities and trunk. Rarely, it coexists with systemic lupus erythematosus. Eosinophilic fasciitis mimics scleroderma, making early diagnosis crucial. Its association with paraproteinaemia necessitates prompt recognition and treatment. Here, we report the case of a 56-year-old female with systemic lupus erythematosus in remission with methotrexate and hydroxychloroquine who presented with a 1-month history of sudden-onset, progressive skin tightening of the extremities and trunk. There was no history of Raynaud's phenomenon, digital ischaemia, or contractures. Based on histopathology and magnetic resonance imaging findings, eosinophilic fasciitis was diagnosed. She responded significantly to treatment with glucocorticoids and mycophenolate mofetil. This case adds to the evidence of eosinophilic fasciitis in lupus. The diagnosis was based on clinical, imaging, and biopsy findings, emphasizing its link to other connective tissue disorders. Future research should explore larger datasets and innovative treatments.

A 46-year-old man was diagnosed with anti jo-1 antibody-positive dermatomyositis 11 years ago and had been treated with prednisolone and tacrolimus. In the present case, after contracting SARS-CoV-2 virus infection, his dyspnoea rapidly worsened, and he presented with renal and cardiac failure. Based on the biopsy results of the same area and anti-U1-RNP antibody positivity, he was diagnosed with systemic sclerosis and scleroderma renal crisis and required haemodialysis. A renal biopsy performed later showed tubular atrophy, intratubular cell debris, and endothelial cell damage, consistent with scleroderma renal crisis. Although rapid skin hardening and high-dose glucocorticoid use are known risks for scleroderma renal crisis, scleroderma renal crisis triggered by novel SARS-CoV-2 virus infection has never been reported before and is very rare. It is crucial to identify the relationship between the scleroderma renal crisis and SARS-CoV-2 virus infection. This relationship can be explained through the RAS system, which is believed to play a role in the development of both.

The patient was a 70-year-old woman. In July 2018, she developed pneumocystis pneumonia and was diagnosed with human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome. Antiretroviral therapy was continued, the HIV-RNA load was suppressed, and the CD4+cells count was maintained. In 2024, the polyarticular pain and swelling persisted. HIV-associated arthropathy, reactive arthritis, and other diseases were excluded. The patient was diagnosed with rheumatoid arthritis (RA) according to the ACR/EULAR 2010 Rheumatoid Arthritis Classification Criteria. Joint radiography revealed narrowing of the wrist joint, and joint ultrasonography showed synovial thickening and power Doppler signals, supporting the diagnosis of RA. Methotrexate was initiated, and remission was achieved and maintained. After starting methotrexate, HIV-RNA load increased transiently but rapidly decreased after that. CD4+cells count was maintained. Patients with HIV have underlying immune dysfunction, and RA requires treatment with immunosuppressants, which makes treatment challenging. Recently, HIV infection has been considered a factor that makes the diagnosis of RA difficult. When symptoms suggestive of RA are observed in HIV-infected patients, it is important to make a thorough differential diagnosis and determine a treatment plan based on the characteristics of RA complicated by HIV infection.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotising vasculitis affecting small vessels accompanied by eosinophilic inflammation. Biological therapies, particularly anti-interleukin-5 (IL-5) monoclonal antibodies, have been shown to be effective in treating refractory EGPA. Mepolizumab, an anti-IL-5 monoclonal antibody, has been approved in Japan for the treatment of EGPA and has a significant glucocorticoid-sparing effect. Benralizumab, an anti-IL-5 receptor monoclonal antibody, has also been reported to reduce the glucocorticoid dose in patients with EGPA. However, several investigators have reported the development of EGPA during biologic treatment. Herein, we present a case of development of refractory EGPA under benralizumab treatment. Although the initial treatment with high-dose glucocorticoids and the administration of benralizumab were temporally effective, the patient's condition did not improve, and the eosinophil count reelevated. After switching benralizumab to mepolizumab, the patient's condition improved, and remission was achieved. Our report suggested that mepolizumab may be an effective treatment option for refractory EGPA after failure of benralizumab treatment.

Lupus podocytopathy (LP) is an increasingly recognised and histopathologically distinct entity within the spectrum of lupus nephritis (LN). It is defined by diffuse podocyte foot process effacement in the absence of subendothelial or subepithelial immune complex deposition and often mimics minimal change disease or focal segmental glomerulosclerosis. LP is typically observed at the onset of systemic lupus erythematosus (SLE) or in patients with known LN. We report a rare case of LP in a 28-year-old Japanese woman with a 12-year history of SLE and no prior renal involvement. She presented with fever, malar rash, arthralgia, and progressive bilateral lower extremity oedema. Laboratory studies revealed marked hypoalbuminemia (1.5 g/dl), nephrotic-range proteinuria (15 g/gCr), elevated anti-dsDNA antibody titers (>400 IU/ml), and hypocomplementemia. Renal biopsy revealed ISN/RPS 2018 class II LN, with mild mesangial hypercellularity and mesangial deposition of IgG, C3, and C1q, without subendothelial or subepithelial immune complexes. Electron microscopy confirmed extensive foot process effacement, establishing the diagnosis of LP. The coexistence of class II LN and LP accounted for the acute onset of nephrotic syndrome in the absence of proliferative changes. The patient was treated with methylprednisolone pulse therapy, high-dose corticosteroids, and intravenous cyclophosphamide, followed by oral cyclosporine. This regimen resulted in prompt clinical and immunological improvement, including near-complete resolution of proteinuria. She remained in remission throughout a 6-month follow-up. This case emphasises the need to consider LP in SLE patients with nephrotic syndrome, even in the absence of prior renal complications during long-term follow-up.