Internal medicine
-
IgG4-related membranous nephropathy (MN) is often refractory to glucocorticoid (GC) therapy, and treatment remains unclear. We herein report a 67-year-old Japanese man with IgG4-related MN and tubulointerstitial nephritis. A post-gastroscopy antibody test revealed Helicobacter pylori infection. ⋯ However, remission proved challenging to achieve, with persistent proteinuria present at 1.0-2.0 g/gCr. We performed multitarget therapy for refractory IgG4-related MN, achieving proteinuria remission (<0.3 g/gCr). Multitarget therapy with low-dose GCs can resolve refractory IgG4-related MN through remission induction of proteinuria and minimize the risks associated with GC therapy.
-
Case Reports
Subcutaneous Panniculitis-like T-cell Lymphoma Lacking Subcutaneous Tumor Mimicking Adult-onset Still's Disease.
We herein report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) resembling adult-onset Still's disease (AOSD). A 40-year-old woman presented with a fever, erythema, and painful subcutaneous nodules on the trunk. Laboratory data and a bone marrow analysis showed hemophagocytic syndrome. ⋯ She was refractory to combination chemotherapy but achieved durable remission with cyclosporine monotherapy. Genetic testing revealed a homozygous HAVCR2 c.245A>G variant (rs184868814) that had caused NLRP3 inflammasome activation. SPTCL and AOSD share a pathogenesis in terms of NLRP3 inflammasome activation, so the clinical phenotype of SPTCL reasonably mimics AOSD.
-
The number of patients with syphilis has been rapidly increasing. Without treatment, syphilis can damage various organs and become life-threatening. We herein report a 29-year-old woman diagnosed with neurosyphilis, acute hydrocephalus, syphilitic uveitis combined with hypertensive retinopathy, and malignant hypertensive nephropathy. ⋯ Neurosyphilis was successfully treated with intravenous penicillin G, and severe hypertension subsequently resolved. However, delayed medical examinations and complications of syphilitic uveitis and hypertensive retinopathy resulted in irreversible visual loss. To prevent irreversible organ damage, early treatment is essential.
-
Exostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren's syndrome. ⋯ Although he did not fulfill the criteria of clinical systemic lupus erythematosus (SLE), he met a stand-alone renal criterion of the Systemic Lupus International Collaborating Clinics (SLICC) 2012. Whether or not a stand-alone renal criterion with EXT1/EXT2 positivity, as in the present patient, can efficiently guide decisions regarding the diagnosis and treatment of SLE remains a clinical dilemma.
-
Aortitis is a rare adverse event associated with granulocyte colony-stimulating factor (G-CSF). Contrast-enhanced computed tomography (CECT) is widely used to diagnose G-CSF-associated aortitis. However, the usefulness of gallium scintigraphy for the diagnosis of G-CSF-associated aortitis is unknown. ⋯ During the diagnosis, gallium scintigraphy revealed hot spots on the arterial walls that appeared inflamed on CECT. Both the CECT and gallium scintigraphy findings disappeared. Gallium scintigraphy can be a supportive diagnostic tool for G-CSF-associated aortitis, especially in patients with an impaired renal function or allergy to iodine contrast.