Systemic lupus erythematosus with C1q deficiency: treatment with fresh frozen plasma

Lupus ◽  
2017 ◽  
Vol 27 (1) ◽  
pp. 134-138 ◽  
Author(s):  
Z Ekinci ◽  
K Ozturk
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Fresh Frozen Plasma ◽  
Complement C3 ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Cutaneous Manifestations ◽  
Fresh Frozen ◽  
C1q Deficiency ◽  
Frozen Plasma

Treatment and outcome of systemic lupus erythematosus (SLE) in C1q deficient patients are rarely reported. The aim of this report is to share our experience about the course of management of three cases diagnosed as SLE with C1q deficiency, in light of present literature. Initial and dominant complaints of three cases from two different families were cutaneous manifestations. One patient was also diagnosed with arthritis and thrombocytopenia. Antinuclear antibody was positive in all cases, whereas anti-dsDNA was negative with normal levels of complement C3, C4 and decreased CH50 activity. C1QA gene of two patients had homozygous nonsense mutation (c.622 > T/p.Gln208Ter). Previously, all of them had been treated with steroids, hydroxychloroquine and methotrexate or azathioprine. It was learned that they had responded only to high dosage prednisolone and their symptoms flared up during dosage reduction even under methotrexate or azathioprine. All symptoms of all three cases improved by daily fresh frozen plasma (FFP) infusions, and once cutaneous lesions subsided, the infusions were reduced to a frequency that would prevent the flare up of the symptoms. Literature search revealed seven reports on fresh frozen plasma treatment in SLE with C1q deficient patients. In this report, it is concluded that severe cutaneous lesions, as seen in these C1q deficient SLE patients, cannot be controlled with conventional immunosuppressive treatment. Instead, regular fresh frozen plasma infusions are proposed as a more reasonable method of treatment.

scholarly journals Spinal and cerebral hematoma in systemic lupus erythematosus and antiphospholipid syndrome: is drug interaction the culprit?

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Emine Duran ◽  
Emre Bilgin ◽  
Ertuğrul Çağrı Bölek ◽  
Oğuzhan Fırat ◽  
Elif Bulut ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Fresh Frozen Plasma ◽  
Systemic Lupus ◽  
Herbal Products ◽  
Spinal Subdural Hematoma ◽  
Anticoagulant Drug ◽  
Fresh Frozen ◽  
Cerebral Hematoma

Abstract Objectives Thrombotic events are common in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Warfarin is the most commonly used anticoagulant drug for thrombosis treatment, but it is can interact with many drugs, foods, or medicinal herbs. Herein, we presented a case with SLE and APS who was complicated by spinal and cerebral hematoma as a result of warfarin interaction. Case presentation Spinal subdural hematoma and frontal intraparenchymal hematoma were occurred in our patient, who was in remission for 2 years with rituximab, hydroxychloroquine and warfarin. We learned that she had been using some herbal products (shepherd’s purse and horsetail) and phenyramidol for a few days. Spinal and cerebral hematomas caused by the interaction of phenyramidol and warfarin were treated with fresh frozen plasma and vitamin K without the need for surgery. Conclusions The drug interactions with warfarin can cause fatal hemorrhagic or thrombotic events. Especially, the patients with SLE and/or APS using warfarin should be warned not to use different medications or herbal agents.

scholarly journals Complement deficiency in pediatric-onset systemic lupus erythematosus

2018 ◽  
Vol 10 (02) ◽  
pp. 232-236 ◽  
Author(s):  
Parisa Afzali ◽  
Anna Isaeian ◽  
Peyman Sadeghi ◽  
Bobak Moazzami ◽  
Nima Parvaneh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Musculoskeletal Symptoms ◽  
Complement C3 ◽  
Complement Deficiency ◽  
Control Group ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Pediatric Onset ◽  
Onset Systemic Lupus Erythematosus

Abstract BACKGROUND: Pediatric-onset systemic lupus erythematosus (pSLE) accounts for about 10%-20% of all patients with SLE. Deficiencies in early complement components of the classical pathway are the strong genetic risk factor for the development of SLE. In this study, clinical and laboratory manifestations of both complement-deficient and normal complement pSLE patients were compared. MATERIALS AND METHODS: To investigate clinical and immunological manifestations of pSLE in Iran, 36 consecutive pSLE patients (onset before 18 years) who were followed up over a period of 2 years, were studied. Complement C1q and C2 levels were measured using radial immunodifusion assay and complement C3 and C4 levels were measured using nephelometry. Medical records were retrospectively evaluated from patient database of Children Medical Center Hospital. Data were assessed through descriptive analysis (confidence interval = 95%), paired t-test, and Pearson correlation test. RESULTS: Twenty-one patients (58%) had at least one component of complement deficiency. Ten patients (27%) had low C1q level, 11 patients (30.5%) had low C2, nine patients (25%) had low C3, and four patients (11%) had low C4 level. Serum level of complement in pSLE was significantly lower than the control group, except C4 (P = 0.005). The low C1q patients had an earlier age of onset of disease (P < 0.0001). The cutaneous manifestations were more frequent and much more severe in pSLE with low complement (100% vs. 73%). The frequency of renal and musculoskeletal symptoms was equal, but renal morbidity was more common in pSLE with low complement. Positivity for anti-ds-DNA was less common in pSLE with low complement (71% vs. 86%). CONCLUSION: In pSLE patients with early disease onset and more aggressive SLE manifestations and negative anti-ds-DNA test, complement deficiency should be considered.

scholarly journals Systemic Lupus Erythematosus Associated with Neurological Manifestations in a Border Collie Bitch

2021 ◽  
Vol 49 ◽  
Author(s):  
Isabel Rodrigues Rosado ◽  
Julia Perinotto Picelli ◽  
Juliana Gonzaga da Silva ◽  
Marina Cazarini Madeira ◽  
Taís Teixeira Zambarda ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Urinary Incontinence ◽  
Lupus Erythematosus ◽  
Clinical Signs ◽  
Neurological Manifestations ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Cutaneous Manifestations ◽  
Immune Mediated ◽  
History Of

Background: Systemic lupus erythematosus (SLE) is an immune-mediated and multisystemic disorder which etiology is believed to be multifactorial. Its clinical signs vary accordingly to affected organs, cutaneous lesions being the most frequently observed. There are few reports of SLE in dogs with neurological manifestations. Therefore, the aim of this report is to describe a case of SLE in a dog with indicative signs of nervous system involvement.Case: A 6-year-old Border Collie bitch was referred to the Veterinary Hospital (HVU) of the University of Uberaba (UNIUBE) with a history of  with cluster seizures, inappetence and urinary incontinence. Erythema and flaking of nasal plan were noted on physical examination, and splenomegaly on abdominal palpation. Thrombocytopenia and slightly increased ALT were found on blood tests. Ehrlichiosis was suspected and doxycycline was prescribed together with phenobarbital for the control of seizures. In the follow-up visit, the dog was still presenting urinary incontinence, thrombocytopenia and splenomegaly. Also, an ulcer on the nasal mucocutaneous junction was observed. The patient went through a neurological examination which indicated thalamocortical lesion. Cerebrospinal fluid samples were obtained for cytology, culture and canine distemper test, and serology tests for leishmaniasis, toxoplasmosis and neosporosis were done. No alterations were found in these exams. The histopathology of the nasal lesion was proceeded and showed results consistent with lupus erythematosus. It was prescribed a 15-day course of prednisolone at immunosuppressive dose. The patient showed clinical improvement with this treatment. Azathioprine was started along with gradual removal of prednisolone. After twenty days of discontinuation of this drug, the dog presented epileptic seizures, urinary incontinence, thrombocytopenia, increased ALT and worsened nasal lesion. Prednisolone at immunosuppressive dose was reintroduced and the dose of azathioprine, increased. One week past this, the patient showed inappetence and an extensive hematoma in the thoracic region. Lab exams confirmed drug-induced acute pancreatitis. All medications were interrupted, the patient was hospitalized, and treatment for pancreatitis was initiated, but the dog passed away.Discussion: For involving multiple body systems and for presenting varied clinical signs, diagnosing SLE can be challenging in clinical routine. The dog from this report was a Border Collie; this breed is considered to be predisposed to this disease. The animal had a history of being exposed to solar radiation for a large part of the day, had dyspigmentation of nasal plan and had no application of sunscreen, predisposing the occurrence of SLE. Neurological signs are uncommon in SLE, but the seizures and the urinary incontinence were the main reasons for the dog’s guardian to look for medical assistance. The suspicion for SLE was raised due to cutaneous manifestations and persistent thrombocytopenia along with splenomegaly. Histopathological findings are essential for diagnosing SLE, as well as antinuclear antibody tests. Nonetheless, due to financial limitations, this last test was not performed. Azathioprine is an immunomodulating drug largely used along with glucocorticoids when treating SLE; however, this medication is prone to induce side effects as the ones presented by the dog from this report. Therefore, it is concluded that SLE should be considered as a differential diagnosis in patients showing cutaneous, hematological, systemic and neurological manifestations, considering the variety of signs caused by this disorder.Keywords: seizures, dog, immune-mediated encephalopathy. Lúpus eritematoso sistêmico associado a manifestações neurológicas em cadela da raça Border CollieDescritores: crises epilépticas, cão, encefalopatia imunomediada.

scholarly journals Cutaneous Manifestations of Systemic Lupus Erythematosus

1970 ◽  
Vol 8 (2) ◽  
pp. 44-48 ◽  
Author(s):  
Nahid Parveen ◽  
Dipak Kumar Das ◽  
FRM Mahbubul Haque ◽  
Mahamudun Nabi Mohammad Momtazul Haque
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Diagnostic Tools ◽  
Systemic Lupus ◽  
Prognostic Information ◽  
Cutaneous Lesions ◽  
Multisystem Disease ◽  
Cutaneous Manifestations ◽  
American Rheumatism Association ◽  
Palmar Erythema

Systemic lupus erythematosus (LE) is a heterogeneous, multisystem, autoimmune disease characterized by the production of auto-antibodies against several cell constituents. The skin is one of the target organs most variably affected by the disease can be yield valuable diagnostic & prognostic information. The present study was conducted to evaluate cutaneous manifestations of SLE in Bangladeshi patients. 100 cases of SLE were collected from the Department of Dermatology, BSMMU, Dhaka. The diagnosis was based on American Rheumatism Association Criteria. Cutaneous changes were recorded on a predevised pro forma. LE specific cutaneous changes noted were: Malar rash or butterfly rash (70.0%), maculopapular rash (19.0%), discoid rash (10.0%), and bullous LE (10.0%). Non specific LE lesions noted were: Photosensitivity (75.0%), alopecia (47.0%), oral mucosal lesion (34.0%), hyperpigmentation (20.0%), nail change (15.0%), Raynauds phenomenon (12.0%), Palmar erythema, genital lesion, periungual talangiectesia, leg ulcers, livedo reticularis, erythema multiform were rare. Systemic lupus erythematosus is a multisystem disease that predominantly affects women. Cutaneous lesions are the most important diagnostic tools as reflected by the fact that they account for four of the 11 revised American Rheumatism Association criteria of SLE. The pattern of skin changes may vary from place to place. Key Words: SLE, LE specific and non specific cutaneous lesions   DOI = 10.3329/jom.v8i2.1405 J MEDICINE 2007; 8 : 44-48

Indeterminate cell histiocytosis in a patient with systemic lupus erythematosus and antiphospholipid antibody syndrome: an unusual association

Lupus ◽  
2019 ◽  
Vol 29 (1) ◽  
pp. 74-78
Author(s):  
N Akdogan ◽  
C Demircan ◽  
E C Bolek ◽  
O Gokoz ◽  
A Karaduman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Factor Xiiia ◽  
Positive Staining ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Cutaneous Manifestations ◽  
First Case

Indeterminate cell histiocytosis (ICH) is an extremely rare clonal proliferative disorder of dendritic cells which presents with skin lesions in the majority of cases. Although extra-cutaneous manifestations are very rare, ICH may involve the mucosa, cornea, and conjunctiva as well as the visceral organs. Since the clinical appearance of cutaneous lesions of ICH is not distinctive, it is diagnosed with histopathological and immunohistochemical findings after clinical suspicion. Herein, we report a 27-year-old man with a two-year history of asymptomatic reddish papules and papulonecrotic lesions on his face, arms and buttocks. He was previously diagnosed with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS), and he had been treated with hydroxychloroquine and low-dose aspirin. Diffuse dermal infiltration of a mixture of histiocytes and lymphocytes accompanied with multinuclear giant cells, the positive CD68 and Factor XIIIa and negative Langerin immunoreactions, along with the positive staining with CD1a and S100, led us to the diagnosis of ICH. To the best of our knowledge, this is the first case of ICH associated with SLE and APS.

scholarly journals Cutaneous Manifestations of Systemic Lupus Erythematosus

2012 ◽  
Vol 2012 ◽  
pp. 1-15 ◽  
Author(s):  
Luís Uva ◽  
Diana Miguel ◽  
Catarina Pinheiro ◽  
João Pedro Freitas ◽  
Manuel Marques Gomes ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Optimal Care ◽  
Cutaneous Manifestations ◽  
American College Of Rheumatology

Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient.

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