Membranoproliferative Glomerulonephritis and Mixed Cryoglobulinemia as a Form of Presentation of Visceral Leishmaniasis

Romero, Maite Padrón; Ribó, Mercedes M Acevedo; Hormigos, Francisco Javier Ahijado; Crespo, Francisco Díaz; Bravo, Laura Cueto; Corredor, Cristina Herraiz; Rojo, Maria Angeles Fernandez; Izquierdo, Rafael Díaz-Tejeiro

doi:10.12659/ajcr.921445

Cited by 3 publications

(1 citation statement)

References 18 publications

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“…However, to date, few case reports of VL during autoimmune hepatitis have been published [7]. While most cases described episodes of VL mimicking autoimmune hepatitis or other autoimmune diseases in which multisystemic and cardiovascular involvement may represent a chief complaint [8][9][10][11][12][13]. Indeed, differential diagnosis is not always trivial, specifically when liver diseases are coexisting, as in this case [14][15][16][17].…”

Section: Discussionmentioning

confidence: 99%

A Challenging Case of Visceral Leishmaniasis

Solimando

Coniglio

Desantis

et al. 2022

Reports

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The term leishmaniasis includes multiple clinical syndromes: visceral, cutaneous, and mucosal leishmaniasis, resulting from an infection of macrophages throughout the reticuloendothelial system in the dermis and the naso-oropharyngeal mucosa, respectively. The clinical phenotype is mainly driven by the leishmania biologic characteristics and, ultimately, also by the host immune status. The disease is endemic in focal areas in the tropics, subtropics, and southern Europe, transmitted by the bite of female phlebotomine sandflies. Sandflies regurgitate the parasite’s flagellated promastigote stage into the host’s skin; promastigotes bind to receptors on macrophages are phagocytized and transformed within phagolysosomes into non-flagellated amastigotes which replicate and infect additional macrophages. Amastigotes ingested by sandflies transform back into infective promastigotes. Depending on the host’s innate and acquired immune status, systemic and visceral leishmaniasis can be characterized by irregular fever, weight loss, enlargement of the spleen and liver, and anaemia. We present a 42 year-old man with long-lasting type 1 autoimmune hepatitis under immunosuppressive treatment. In January 2017, the patient started to experience low-grade unresponsiveness to empiric antibiotic therapy. The patient developed severe anemia and progressive multilineage cytopenia accompanied by increased levels of inflammatory markers. FDG-PET revealed increased glucose uptake in the liver, spleen, and the whole bone marrow. The subsequently performed bone marrow biopsy evidenced Leishmania amastigotes inside macrophages, confirmed by serological positivity to anti-Leishmania antibody. Immunosuppressive therapy was suspended and replaced by treatment with amphotericin B at 4 mg/kg/day from day 1 to day 5, followed by a single infusion on days 10, 17, 24, 31, and 38. The bone marrow smear after treatment still evidenced few Leishmania amastigotes; in consideration of the patient’s immunosuppression status, two further doses of amphotericin B on days 45 and 52 were employed, leading to infection resolution. In real-life, as exemplified in this case, administering two additional doses of amphotericin B (concerning the guidelines) offered an additional therapeutic opportunity for a patient under long-term immunosuppressive treatment.

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Section: Discussionmentioning

confidence: 99%

A Challenging Case of Visceral Leishmaniasis

Solimando

Coniglio

Desantis

et al. 2022

Reports

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Parasitic Infection and Autoimmunity

Ling,

Abu-Shakra

2024

Infection and Autoimmunity

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Nonviral cryoglobulinemic vasculitis: an updated review for clinical practice

Núñez-Conde,

Rodríguez-Pintó,

Alba-Garibay

et al. 2023

Vessel Plus

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The clinical spectrum of cryoglobulinemic-associated diseases is broad and heterogeneous, with manifestations ranging from mild symptoms (e.g., isolated palpable purpura) to organ- and life-threatening involvement (e.g., membranoproliferative glomerulonephritis). Cryoglobulins are classified into three types. Type I cryoglobulinemia consists of one monoclonal immunoglobulin (Ig) and is practically always associated with B-cell lymphoproliferative disorders. In contrast, type II/III (mixed) cryoglobulinemia is composed of mono- or polyclonal IgM with rheumatoid factor activity bound to polyclonal IgG. Since the introduction of more efficient therapies for chronic hepatitis C virus (HCV), other diseases such as systemic autoimmune disorders and lymphoproliferative neoplasms have been established as the main causes of mixed cryoglobulinemic vasculitis. The pathogenesis of cryoglobulinemic vasculitis is a complex multifactorial process that involves B-cell aberrant lymphoproliferation and autoantibody production. Therefore, treatment of these patients may involve not only measures aimed to mitigate the severity of clinical manifestation but also those that address the associated underlying disease responsible for Ig production. The treatment of patients with type I cryoglobulinemia is primarily focused on controlling B lymphocyte clones responsible for cryoglobulin production, mostly with chemotherapy drugs. Treatment of mixed cryoglobulinemia syndrome is based on rituximab plus glucocorticoids, which induces remission in the vast majority of cases. In the rare patients that do not respond to rituximab administration, potential rescue approaches include alkylating agents, biologic therapies, conventional immunosuppression, and plasma exchange, although with partial efficacy. This narrative review explores the etiology, pathophysiology, clinical manifestations, treatment, and prognosis of nonviral cryoglobulinemic disease. A special focus is placed on the treatment of type I cryoglobulinemia and rituximab-resistant non-HCV cryoglobulinemic vasculitis.

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Membranoproliferative Glomerulonephritis and Mixed Cryoglobulinemia as a Form of Presentation of Visceral Leishmaniasis

Cited by 3 publications

References 18 publications

A Challenging Case of Visceral Leishmaniasis

A Challenging Case of Visceral Leishmaniasis

Parasitic Infection and Autoimmunity

Nonviral cryoglobulinemic vasculitis: an updated review for clinical practice

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