The Diagnostic Conundrum of Glomerular Crescents With IgA Deposits.

Introduction: Glomerulonephritis (GN) with crescents and IgA deposits in kidney biopsy poses a frequent diagnostic and therapeutic dilemma because of multiple possibilities. Methods: Native kidney biopsies showing glomerular IgA deposition and crescents (excluding lupus nephritis) were identified from our biopsy archives between 2010 and 2021. Detailed clinicopathologic features were assessed. One-year clinical follow-up on a subset of cases was obtained. Results: A total of 285 cases were identified, and these clustered into IgA nephropathy (IgAN, n = 108), Staphylococcus or other infection-associated GN/infection-related GN (SAGN/IRGN, n = 43), and antineutrophil cytoplasmic antibody-associated GN (ANCA-GN, n = 26) based on a constellation of clinicopathologic features, but 101 cases (group X) could not be definitively differentiated. The reasons have been elucidated, most important being atypical combination of clinicopathologic features and lack of definitive evidence of active infection. Follow-up (on 72/101 cases) revealed that clinicians' working diagnosis was IgAN in 43%, SAGN/IRGN in 22%, ANCA-GN in 28%, and others in 7% of the cases, but treatment approach varied from supportive or antibiotics to immunosuppression in each subgroup. Comparing these cases as "received immunosuppression" versus "non-immunosuppression," only 2 features differed, namely C3-dominant staining, and possibility of recent infection (both higher in the no-immunosuppression group) (P < 0.05). Renal loss was higher in the non-immunosuppression subgroup, but not statistically significant (P = 0.11). Conclusion: Diagnostic overlap may remain unresolved in a substantial number of kidney biopsies with glomerular crescents and IgA deposits. A case-by-case approach, appropriate antibiotics if infection is ongoing, and consideration for cautious immunosuppressive treatment for progressive renal dysfunction may be needed for best chance of renal recovery.

Felty's syndrome as an initial presentation of rheumatoid arthritis: a case report.

INTRODUCTION: Felty's syndrome is an uncommon but severe extra-articular manifestation of rheumatoid arthritis. Felty's syndrome is characterized by the triad of rheumatoid arthritis, neutropenia, and splenomegaly. The lifetime risk of Felty's syndrome for a rheumatoid arthritis patient is less than 1% and there are only few case reports of Felty's syndrome with neutropenia preceded clinical evidence of arthritis. We present a case which is atypical presentation of Felty's syndrome without arthritis. CASE PRESENTATION: We present a case of 31-year-old man who presented with fever and skin infection, found to have neutropenia. The work up showed splenomegaly and other evidences support Felty's syndrome diagnosis without arthritis presentation. CONCLUSION: Patients with unexplained, continuous neutropenia without arthritis but with high level of rheumatoid factor and positive antibodies to cyclic citrullinated peptides should be suspected of developing Felty's syndrome as an initial presentation of rheumatoid arthritis.