Silent Storm Unveiled: Lupus Nephritis and Cerebral Vasculitis in Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder known for its intricate clinical manifestations, spanning a spectrum of symptoms, including neuropsychiatric SLE (NPSLE) and lupus nephritis (LN). This condition predominantly affects young women of childbearing age, presenting a diverse array of symptoms that pose challenges in both diagnosis and treatment. Diagnosing central nervous system (CNS) involvement in SLE remains notably difficult despite being rooted in an autoimmune inflammatory response driven by cytokine surges. There exists no single definitive test for diagnosis, necessitating a thorough evaluation of clinical presentations, neurological indicators, and specific antibody detection. LN typically evades diagnosis until the patient progresses to a state of advanced renal insufficiency, demanding aggressive therapeutic interventions. In this discourse, we examine a case marked by generalized tonic-clonic seizures. While epilepsy might be initially suspected, in this instance, the underlying cause lay deeper, concealed within the complexities of autoimmune dysregulation. Additional symptoms included generalized edema, sun-exposed rash, oral ulcers, and recurrent fever over the past six months. The puzzle pieces eventually coalesced through meticulous examination of each clinical manifestation, coupled with laboratory analyses, neuroimaging studies, and renal biopsy, revealing a complex scenario of cerebral vasculitis concurrent with LN in a case of SLE.

A Rare Case of Atypical Anti-glomerular Basement Membrane Disease.

Anti-glomerular basement membrane (Anti-GBM) disease is a severe form of glomerulonephritis (GN) that predominantly impacts individuals aged 20 to 70. It arises from the presence of circulating antibodies that specifically target an antigen inherent to the basement membranes of glomerular and alveolar structures. A unique subset within this category is termed atypical anti-GBM disease. In this variant, a distinctive feature is the widespread linear staining of the glomerular basement membrane (GBM) by IgG observed through immunofluorescence microscopy, with the notable absence of anti-GBM antibodies in the patient's serum. Here, we present an unusual case involving a 65-year-old female patient who sought medical attention due to rapidly progressing renal failure. The initial management included six hemodialysis sessions. Following a kidney biopsy, the diagnosis revealed a sclerosed phase of diffuse crescentic glomerulonephritis, attributed to atypical anti-GBM disease. Given the presence of diffuse crescents on the kidney biopsy, the medical team opted for an aggressive treatment regimen, commencing with intravenous methylprednisolone, followed by oral cyclophosphamide and oral prednisolone. Plasmapheresis was also recommended as part of the treatment plan, although it did not materialize due to the family's reluctance. Despite exhaustive efforts, the renal failure exhibited no signs of improvement, leading to the patient's discharge with a plan for ongoing maintenance hemodialysis. It is crucial to emphasize the pivotal role of immunosuppressive medications in managing this condition, as they play a critical role in preventing antibody formation and subsequent hypersynthesis that can exacerbate the disease.

Nephrotic Syndrome in a Retroviral Disease Due to AA Amyloidosis: A Rare Presentation.

Kidney disease poses a significant burden on individuals with HIV infection. In the pre-ART era, HIV-associated nephropathy (HIVAN) was the most common renal pathology identified in individuals with HIV. However, the widespread use of ART has led to changes in the spectrum of renal pathologies associated with HIV. HIV infection is an unclear cause of AA amyloidosis. Here, we report a rare case of an HIV-positive patient presenting with nephrotic syndrome which turned out to be AA amyloidosis on renal biopsy.

Lupus Nephritis With Collapsing Glomerulopathy: A Rare Association.

Lupus nephritis (LN) is one of the most severe organ manifestations of systemic lupus erythematosus (SLE). Crescentic lupus nephritis rarely presents as rapidly progressive renal failure (RPRF) and needs prompt initiation of treatment. Collapsing glomerulopathy (CG) itself is associated with poor renal survival. Collapsing glomerulopathy's association with lupus nephritis is rarely reported in the literature. It may indicate a severe form of lupus podocytopathy.

Unusual case of coexistent pulmonary cryptococcosis and tuberculosis in an immuno-competent host.

Coexistence of pulmonary cryptococcosis with other infections has commonly been described in immuno-suppressed individuals. In immuno-competent hosts, such coexistence is rare and mostly described in disseminated disease or uncommonly involving different sites. The simultaneous coinfection of cryptococcosis and tuberculosis of lung in an immuno-competent host is extremely rare with only one previously reported case in the literature. This is the second such case and the first to be reported in India. We describe a case of a 36-year-old immuno-competent male who presented with haemoptysis and cough. Computed tomography showed a sub-pleural lung nodule. Diagnostic thoracoscopic wedge resection of the right lung nodule revealed granulomatous inflammation with cryptococcus on histopathology. Coexistent tuberculosis was diagnosed by microbiological culture study on lung tissue. The patient responded clinically to fluconazole and anti-tubercular therapy. This case shows that although rare, coexistent infections can occur in immuno-competent persons and highlights the importance of careful evaluation and tissue microbiological culture examination.

Sclerosing angiomatoid nodular transformation of spleen masquerading as carcinoma breast metastasis: Importance of splenic biopsy in obviating splenectomy.

Metastasis to spleen is rare and usually occurs in the setting of extensive multivisceral metastatic disease. A 60-year-old lady with appropriately managed early breast cancer (breast conservative surgery for Grade-2 infiltrative ductal carcinoma [0.7 cm × 0.5 cm diameter]) in 2006, was detected to have splenic incidentaloma (1.4 cm × 0.8 cm) in November 2012, which was fluoro-deoxy-glucose (FDG) avid on positron emission tomography. Fine needle spiration cytology (FNAC) was normal. More than doubling of lesion size by March 2015 (3.83 cm × 3.03 cm diameter) with persistent FDG positivity lead to Tru-Cut biopsy of spleen, which revealed multiple nodular areas of congestion and hemorrhage, composed of sinusoids (CD31+, CD8+, and CD34-), capillaries (CD31+, CD8-, and CD34+), and small veins (CD31+, CD8-, and CD34-), fibrosis, around these nodular areas along with numerous histiocytes (CD68+) consistent with diagnosis of sclerosing angiomatoid nodular transformation (SANT) of spleen. SANT is a benign, reactive vascular transformation of spleen, notorious to mimic metastasis, the cause of its 18FDG avidity due to its rich content of macrophages and myofibroblasts, usually diagnosed postsplenectomy. This report highlights the importance of splenic biopsy over FNAC is diagnosing splenic incidentalomas, which can help prevent splenectomy, and hence the associated morbidity. This is the first report of SANT in carcinoma breast mimicking metastasis.

Angiosarcoma Developing in a Vagal Schwannoma: A Rare Case Report.

Angiosarcoma arising in a schwannoma is extremely rare with only fourteen cases having been reported in the literature to the best of our knowledge. Amongst these fourteen, only five cases developed from vagal schwannoma. We describe a case of epithelioid angiosarcoma arising in a long standing vagal schwannoma in a 41 years male patient. Grossly the tumor was well encapsulated with variegated cut surface. On microscopy the tumor had two distinct components composed of benign schwannoma and malignant angiosarcoma which were further confirmed by immunohistochemistry. On further work up, he was found to have multiple distant metastases. This is the sixth reported case of angiosarcoma arising in a vagal schwannoma. The proposed histogenesis of this rare transformation, its prognostic factors and a review of literature regarding this entity is discussed.