Simultaneous Occurrence of Collagen Type III Glomerulopathy and Immunoglobulin A Nephropathy: A Rare Case Report.

BACKGROUND Collagen type III glomerulopathy (CG) is a rare disease with poorly understood pathogenesis, usually identified by abnormal collagen type III accumulation in glomeruli and manifesting as progressive deterioration of kidney function with nephrotic-range proteinuria. Immunoglobulin A nephropathy (IgAN) is the most prevalent glomerulopathy worldwide and is a leading cause of end-stage renal disease as a result of progressive fibrotic changes. Fibrosis is primarily caused by collagen type III deposition, which may explain the simultaneous occurrence of IgAN and CG. CASE REPORT A young man presented with clinical and laboratory evidence of chronic kidney injury, including long-term nephrotic-range proteinuria and microscopic hematuria. Partial improvement in proteinuria was achieved with steroid therapy and conservative management. As the non-invasive workup was inconclusive, and a complete recovery of kidney function was not achieved, a kidney biopsy was done. Histopathological microscopic examination revealed advanced IgA nephropathy, Oxford classification M0E1S1T2C0, with features highly suggestive of type III collagen glomerulopathy. CONCLUSIONS We described a case of collagen type III glomerulopathy, also known as collagenofibrotic glomerulopathy, and its association with concurrent immunoglobulin A nephropathy in a healthy man presenting with chronic proteinuria and microscopic hematuria. As the number of reported cases in the Middle East is rising, we present this report to improve understanding and greater recognition of such cases.

Splenic Infarcts and Pulmonary Renal Syndrome in a Young Patient with Double-Positive Anti-GBM and ANCA-Associated Vasculitis.

Double-positive disease, defined by double-seropositivity for serum anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) is a rare cause of pulmonary-renal syndrome. Here, we present an exceptional course of a 20-year-old male with seropositivity for anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies and anti-GBM antibody, who presented first with renal impairment due to focal necrotizing crescentic glomerulonephritis. After receiving treatment, he presented two years later with a relapse manifesting with diffuse alveolar hemorrhage and multiple splenic infarcts. We discuss the clinical presentation patterns and treatment strategies of this entity.

Renal histopathology spectrum in children with kidney diseases in Saudi Arabia, 1998-2017.

OBJECTIVES: To identify the trends in the diagnostic frequency of glomerular disease subtypes by renal biopsy in children in Saudi Arabia over the last 20 years. METHODS: In this retrospective observational study, we identified all patients aged less than 18 years for whom native kidney biopsy was performed between 1998 and 2017. The period during which biopsy was performed (1998-2004, 2005-2011, and 2012-2017) and the demographic information and their association with the prevalence of various glomerular disease subtypes were our primary outcomes. Results: A total of 326 cases with renal biopsy were analyzed; the mean age of participants being 11 years and 45.4% of them were girls. Unexpectedly, secondary glomerulonephritis accounted for 42.3% of the cases, and lupus nephritis was the most common cause noted in 20.7% of the cases. The minimal change and focal segmental glomerulosclerosis were the most common glomerulonephritis in 59% of the cases. The frequency of membranoproliferative glomerulonephritis and mesangioproliferative glomerulonephritis significantly decreased from 15% and 17% in the period prior to 2004 to 3.3% (p=0.003) and 1.7% in 2012-2017 (p less than 0.001). CONCLUSIONS: We found a considerable shift in the frequency of many glomerular disease subtypes in 1998-2017, which make clinical predication of the underlying etiology challenging for clinician. Renal biopsy still remains a critical diagnostic procedure for managing a considerable proportion of renal diseases.

Low-grade papillary cystadenocarcinoma of the parotid gland: presentation of a case with cytological, histopathological, and immunohistochemical features and pertinent literature review.

Cystadenocarcinoma of the salivary glands is a very rare, slow growing, low-grade malignant neoplasm. However, when it occurs, it poses a diagnostic challenge. They are usually well circumscribed and exhibit multicystic appearance. The cytological features of fine-needle aspirates show bland appearing papillary and micropapillary neoplasm with variable cellularity and absence of cytological atypia. The cells are small to medium in size with abundant and occasionally vacuolated cytoplasm. The nuclei are eccentric, bland appearing, round, and at the most part uniform in size without anaplasia or mitotic activity. The background is commonly mucoid. Histologically, the neoplasm is usually well circumscribed and contains variably sized multicystic spaces with papillary structures. Foci of definite stromal invasion by tumor cells have to be present to justify the diagnosis of carcinoma. The remainders of the cytological features are similar to these seen on cytological smears. Typically, they are slow growing and conservative, but complete surgical excision is considered adequate. We present a case in a 57-year-old Saudi man that we believe the first from our region demonstrating the clinical and pathological features.