Autosomal chronic granulomatous disease and systemic lupus erythematosus with fatal outcome

Progressive multifocal leukoencephalopathy (PML) caused by reactivation of the JC virus (JCV), a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE). We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine) since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions) and JCV isolation from the cerebrospinal fluid (700 copies/ml). IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.

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CLINICAL OBSERVATION OF UNTREATED SYSTEMIC LUPUS ERYTHEMATOSUS WITH INFECTIOUS COMPLICATIONS AND FATAL OUTCOME

Clinical Medicine and Pharmacology ◽

10.12737/2409-3750-2020-6-3-33-36 ◽

2020 ◽

Vol 6 (3) ◽

pp. 33-36

Author(s):

B. Barieva

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Fatal Outcome ◽

Clinical Situation ◽

Infectious Complications ◽

Multiple Organ ◽

Rapid Progression ◽

Systemic Lupus ◽

Diagnostic Difficulties ◽

Omental Bursa

Among rheumatic diseases, a special place is occupied by systemic lupus erythematosus (SLE), this is due to its variety of clinical nonspecific manifestations, which leads to diagnostic difficulties in the early stages.This article presents a clinical case of severe SLE, previously not diagnosed and untreated, with the rapid progression of multiple organ failure (damage to the lungs, cardiovascular system, kidneys), which led to death. Previously, the patient was treated in the oncology and surgical departments with an abscess of the omental bursa and steatopancreonecrosis, later she was hospitalized in the hematology department with three-growth pancytopenia and hemolytic anemia, and from there transferred to the rheumatology department. This clinical situation demonstrates the importance of timely diagnosis, diagnosis and treatment of SLE.

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What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report

Transplantation Proceedings ◽

10.1016/j.transproceed.2017.03.007 ◽

2017 ◽

Vol 49 (4) ◽

pp. 913-915

Author(s):

H. Pinto ◽

R. Leal ◽

L. Rodrigues ◽

L. Santos ◽

C. Romãozinho ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Case Report ◽

Lupus Erythematosus ◽

Fatal Outcome ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Systemic Lupus ◽

Collapsing Glomerulopathy

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Triple Valvulopathy and Jaccoud’s Arthropathy: A Case Report and Literature Review

Case Reports in Rheumatology ◽

10.1155/2016/4147068 ◽

2016 ◽

Vol 2016 ◽

pp. 1-2

Author(s):

Ali Naderi Mahabadi ◽

Bassam Alhaddad ◽

Stanley Ballou

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Heart Valves ◽

Fatal Outcome ◽

Disease Recurrence ◽

Point Of View ◽

Systemic Lupus ◽

Rare Presentation ◽

First Case ◽

History Of

Cardiac involvement is fairly common in patients with systemic lupus erythematosus (SLE). It may involve all layers of the heart and coronary arteries as well as the heart valves. We report an extremely rare presentation of valvulitis and valvular dysfunction associated with systemic lupus erythematosus. This is the first case of lupus valvulitis which required three mechanical prosthetic valve replacements with disease recurrence leading to a fatal outcome. This is, in our point of view, the consequence of aggressive natural history of the disease and perhaps late diagnosis and treatment of underlying SLE which was unsuccessful.

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