Monoclonal gammopathy of undetermined significance combined with renal damage is named monoclonal gammopathy of renal significance. There are few reports about IgA vasculitis in patients with monoclonal gammopathy of undetermined significance. Here, we report a case of monoclonal gammopathy of renal significance, who had manifestations of IgA vasculitis, including purpura, gastrointestinal bleeding and joint pain. The patient had elevated serum creatinine levels, prompting further investigation through immunofixation electrophoresis and bone marrow aspiration biopsy. Immunofixation electrophoresis showed IgA-λ-type monoclonal immunoglobulin, while the bone marrow aspiration biopsy suggested plasmacytosis. Kidney biopsy indicated membranous hyperplastic glomerulonephritis, light and heavy chain deposition, IgA-λ. The patient was diagnosed with monoclonal gammopathy of renal significance. In light of the elevated serum creatinine, the patient was treated with chemotherapy regimen (bortezomib +cyclophosphamide +dexamethasone). After chemotherapy, there was no significant improvement in the patient's renal function. Subsequently, the patient experienced abdominal pain, skin purpura, joint pain and severe gastrointestinal bleeding. Gastroenteroscopy did not find the exact bleeding position. Angiography revealed hyperplasia of left jejunal artery. Surgical operation found that the bleeding site was located between the jejunum and ileum, where scattered hemorrhagic spots and multiple ulcers were present on the surface of the small intestine, with the deepest ulcers reaching the serosal layer. And the damaged intestine was removed during the operation. Intestinal pathology showed multiple intestinal submucosal arteritis, rusulting in intestinal wall necrosis and multiple ulcers. Considering intestinal lesions as gastrointestinal involvement of IgA vasculitis, methylprednisolone was used continually after the operation, and the patient's condition was improved. However, after half a year, the patient suffered a severe respiratory infection and experienced a recurrence of serious gastrointestinal bleeding. It was considered that the infection triggered the activity of IgA vasculitis, accompanied by gastrointestinal involvement. Finally, the patient died from gastrointestinal bleeding. The present case represented a patient with monoclonal gammopathy of renal significance and IgA vasculitis, prominently presenting with renal insufficiency and severe gastrointestinal bleeding, making the diagnosis and treatment process complex. Patients with IgA monoclonal gammopathy who presented with abdominal pain, purpura, and arthralgia should be vigilant for the possibility of concomitant IgA vasculitis. The treatment of cases with IgA vasculitis combined with monoclonal gammopathy of renal significance was rather challenging. Plasma cell targeting therapy might be an effective regimen for IgA vasculitis with monoclonal gammopathy. However, patients with poor renal response to the treatment indicated poor prognosis.
Humans ; Cyclophosphamide/administration & dosage* ; Gastrointestinal Hemorrhage/etiology* ; IgA Vasculitis/complications* ; Immunoglobulin A ; Intestine, Small/pathology* ; Kidney/pathology* ; Kidney Diseases/pathology* ; Monoclonal Gammopathy of Undetermined Significance/complications* ; Necrosis ; Paraproteinemias/complications* ; Vasculitis/etiology*

Rheumatoid arthritis (RA) and myasthenia gravis (MG) are two distinct autoimmune diseases. Compared with the general population, the incidence of RA is notably higher among patients with MG. Similarly, the rate of MG in patients diagnosed with RA is also significantly increased. In this report, we presented an elderly female patient with a history usage of long-term glucocorticoid and conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), whose RA symptoms remained inadequately controlled. She later exhibited drooping of the right eyelid and double vision, leading to a diagnosis of ocular myasthenia gravis (OMG). Then, we made a literature review and found that the RA patients with co-existing MG were relatively more common in middle-aged and elderly women, and most of them did not have thymoma. Thymoma wasn ' t found in our patient, which was consistent with the cli-nical characteristics of RA complicated with MG reported in previous reports. In addition, there was li-mited treatment experience in patients with both RA and MG. The treatment stratergies for RA or MG included glucocorticoids and immunosuppressants. Among the 18 patients we analyzed, 8 patients expe-rienced relief after csDMARDs, while other 8 patients received biologics or targeted DMARDs, including tumor necrosis factor inhibitors (TNFi) in 5 cases, JAK inhibitors in 2 cases, and B-cell depletion therapy (rituximab) in 2 cases. What called for special attention was that one RA patient was diagnosed with MG after using 23 months of methotrexate and 6 weeks of etanercept (TNFi), with rituximab 1 000 mg for the first time, followed by 500 mg every 6 months, and finally both RA and MG were well controlled. For the patient in this study, MG symptoms improved with increased dosage of prednisone. In order to tapper the dose of glucocorticoid, it was necessary for more potent immunosuppressant for both RA and MG. Given her history of cardiac conditions, JAK inhibitors were not considered, and due to the uncertain efficacy of TNFi, we chose to administer low-dose rituximab (100 mg). Subsequent follow-up revealed stable conditions for both RA and MG, allowing for discontinuance of glucocorticoid after 5 months. It reflected the potential efficacy and cost-effectiveness of low-dose, long-interval rituximab in treating RA patients combined with MG, while it also minimized infection risks. However, the duration for subsequent infusions remained uncertain and required further observation. In conclusion, RA combined with MG is rare. For patients exhibiting poor responses to csDMARDs, low-dose, long-interval rituximab might be a promising treatment option.
Humans ; Arthritis, Rheumatoid/drug therapy* ; Rituximab/administration & dosage* ; Myasthenia Gravis/drug therapy* ; Female ; Antirheumatic Agents/administration & dosage* ; Aged ; Glucocorticoids/therapeutic use*

Biopsy, Needle ; Genetic Variation ; Hepatitis B, Chronic ; pathology ; Humans ; Liver ; pathology ; Specimen Handling