Initial Presentation Of Lupus Research Articles

Axonal polyneuropathy as initial presentation of lupus in a 15-year-old male teenager “Case report”

Systemic lupus erythematosus (SLE) is a chronic autoimmune systemic disease, of the connectivitis group. This condition particularly affects young women between the ages of 20 and 40. Several systems are affected during the disease including the nervous system where central damage is more described than peripheral damage. We report here the case of a 15 year old male teenager with systemic lupus erythematosus whose initial clinical manifestation was acute inflammatory axonal polyneuropathy. This diagnosis was made on the basis of allodynia predominantly in gloves and socks with clinical examination of bilateral peripheral neurogenic syndrome predominant in both lower limbs all associated with fever. The electroneuromyogram showed signs of sensory and motor impairment (more marked in sensory) of the axonal type in both lower limbs, compatible with polyneuropathy. The patient developed several skin lesions such as erythematous and scaly patches on the extremities, and purpuric macules of the palms of hands. All the antibodies tested in the blood came back positive and the skin biopsy described a proliferation of vascular capillaries with a fibrous and myxoid wall, dissociated by inflammatory cells, suggesting inflammatory involvement. Under treatment with hydroxychloroquine and corticosteroids, the patient presented a marked improvement in the general condition as well as on the functional level with regression of sensory and motor disorders.

Axonal Polyneuropathy as Initial Presentation of Lupus in a 15-year-old Male Teenager “Case Report”

Systemic lupus erythematosus (SLE) is a chronic autoimmune systemic disease, of the connectivitis group. This condition particularly affects young women between the ages of 20 and 40. Several systems are affected during the disease including the nervous system where central damage is more described than peripheral damage. We report here the case of a 15 year old male teenager with systemic lupus erythematosus whose initial clinical manifestation was acute inflammatory axonal polyneuropathy. This diagnosis was made on the basis of allodynia predominantly in gloves and socks with clinical examination of bilateral peripheral neurogenic syndrome predominant in both lower limbs all associated with fever. The electroneuromyogram showed signs of sensory and motor impairment (more marked in sensory) of the axonal type in both lower limbs, compatible with polyneuropathy. The patient developed several skin lesions such as erythematous and scaly patches on the extremities, and purpuric macules of the palms of hands. All the antibodies tested in the blood came back positive and the skin biopsy described a proliferation of vascular capillaries with a fibrous and myxoid wall, dissociated by inflammatory cells, suggesting inflammatory involvement. Under treatment with hydroxychloroquine and corticosteroids, the patient presented a marked improvement in the general condition as well as on the functional level with regression of sensory and motor disorders.

Diffuse Alveolar Hemorrhage: A Rare Life-Threatening Condition in Systemic Lupus Erythematosus

Diffuse alveolar hemorrhage (DAH) is a rare life-threatening complication in systemic lupus erythematosus (SLE) associated with high mortality rates. DAH is more common in women, and mean age of onset is around 30 years. It mostly occurs in patients with established diagnosis of SLE but can be the initial presentation of lupus in approximately 20%. DAH should be suspected in lupus patient presenting with new pulmonary infiltrates, decline in hemoglobin, hemoptysis, dyspnea, hypoxemia, and increase in carbon monoxide diffusion capacity. Radiographic evidence of bilateral pulmonary alveolar infiltrates that are usually perihilar or basilar with sparing of apices is seen. DAH can often mimic clinically and radiologically severe pneumonia or ARDS. Treatment includes high-dose corticosteroids, cyclophosphamide, and plasmapheresis. We report a case of diffuse alveolar hemorrhage complicating SLE flare-up in a male patient.

Kawasaki disease and juvenile systemic lupus erythematosus

Kawasaki disease (KD) is a common vasculitis in childhood. To the authors' knowledge, only one case of juvenile systemic lupus erythematosus (JSLE)-like onset mimicking KD and another case of KD and JSLE association have previously been described. However, the prevalence of this association of the two diseases was not reported. Therefore, over 27 consecutive years, 5419 patients were followed at the Pediatric Rheumatology Unit and 271 (5%) of them met the ACR classification criteria for JSLE. Two (0.7%) of them were female. These also had KD according to European League against Rheumatism / Paediatric Rheumatology European Society (EULAR/PReS) consensus criteria and are described in this report. One case was a 13-year-old who presented all six KD criteria. Echocardiogram showed pericardial effusion, dilatation and tortuosity of right and left coronary, and her symptoms promptly improved after treatment with intravenous immunoglobulin (IVIG). Lupus diagnosis was established a few days later. Another case was a 4-year-old who had also met all six KD criteria, with improvement after IVIG, and lupus diagnosis was made 1 year later. In conclusion, the frequency of the association between these two autoimmune diseases was rare. The occurrence of a second autoimmune systemic disease in a patient with a history of KD should also be considered. Furthermore, the initial presentation of lupus may mimic KD.

Neutrophilic dermatosis revisited: An initial presentation of lupus?

We report 7 patients with a distinct and unusual eruption consisting of a neutrophilic dermatosis in conjunction with lupus erythematosus (LE). The significance of these findings and their relevance to LE are discussed. We aimed to evaluate the significance of eruptive neutrophilic dermatosis in conjunction with LE. Seven original cases were collected during 10 years at a tertiary referral center, and were reviewed by a single board-certified dermatopathologist. All patient demographics were tabulated and analyzed. Eleven articles reporting 15 similar cases were obtained from a literature review. Of a total of 7 adult patients, 14% (1 of 7) had a history of LE, whereas 86% (6 of 7) exhibited synchronous initial presentation of neutrophilic dermatosis with LE. Of note, 100% (7 of 7) of the patients exhibited cutaneous lesions on sun-exposed sites. Only 7 of 400,000 cases showed this phenomenon, giving rise to the idea this may be just by chance. Our data and literature review suggest the existence of a neutrophilic dermatosis distinct from conventional Sweet syndrome that may herald conventional signs and symptoms or represent an initial presentation of cutaneous LE. This neutrophilic dermatosis may share a similar pathogenic mechanism related to ultraviolet exposure.

Posterior Reversible Encephalopathy Syndrome in Systemic Lupus Erythematosus

To study the clinical profile of posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE) and analyze the risk factors and outcomes associated with it. We identified patients with SLE and PRES from January 2006 to October 2010. Data were collected on demographic details, lupus characteristics, PRES-related features, laboratory abnormalities, treatment details, and outcomes. We studied 13 patients (all female) ages 14-37 years (median 23 yrs; 4 were aged < 18 yrs with juvenile SLE). Duration of lupus ranged from 1.5 to 36 months (median 6 mo). Six patients had PRES as a part of their initial presentation of lupus. All had active lupus and hypertension; 9 had nephritis. Four patients were on treatment with cyclophosphamide therapy when they developed PRES. Antihypertensives and antiepileptics were the mainstay of treatment along with supportive care. Immunosuppressive therapy was guided by lupus-related major organ manifestations. Two patients had focal neurological deficits; one had persistent hemiparesis at followup. One patient died. PRES occurs in young lupus patients and in the early part of the disease. Focal deficits are not uncommon. It can be the presenting manifestation of lupus. Management is predominantly symptomatic. Immunosuppression is directed by other major organ manifestations. Early diagnosis and appropriate management is productive.

Systemic lupus erythematosus presenting as Stevens–Johnson syndrome and toxic epidermal necrolysis: a report of three cases

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatological conditions that are characterized by mucositis, epidermal detachment and erosions. The underlying etiology in SJS and TEN is almost invariably secondary to drugs. Rarely, other causes such as systemic lupus erythematosus (SLE), infections and vaccinations have been implicated. This report describes three patients with SLE who presented with manifestations of SJS/TEN without a clear drug causality. All three patients presented with photodistributed macular exanthema, which evolved to target lesions, bullae, erosions or sheet-like detachment. This was associated with oral mucositis and conjunctivitis. The onset of the rash was insidious with a protracted clinical course. Ultraviolet exposure and steroid tapering appear to be precipitating factors. In two of the patients, SJS and TEN were the initial presentation of lupus. Although SJS and TEN are almost invariably due to medications, they may, rarely, be an initial presentation of lupus, particularly when associated with an initial photodistribution, absence of genital involvement and a prolonged clinical course.

Lupus Mastitis

Lupus mastitis is an uncommon presentation of lupus erythematosus profundus or lupus panniculitis, a rare variant of lupus erythematosus characterized by inflammation of the subcutaneous fat. Lupus mastitis can present as single or multiple subcutaneous or deep breast masses, often clinically mimicking malignancy. Although lupus mastitis is rare, with less than 25 cases reported, the histologic features are distinct. Awareness of the entity and familiarity with the histologic features allow for accurate diagnosis and appropriate patient management. It most commonly affects women with a mean age at diagnosis of 40 years and an age range of 18 to 70 years. Typical histologic findings in lupus mastitis include a lymphocytic lobular panniculitis with plasma cells and hyaline fat necrosis. The lymphocytic infiltrate can be nodular, diffuse, periductal, and/or perilobular and germinal centers can frequently be identified. Lymphocytic vasculitis is also common. Immunohistochemistry shows a mixed T and B-cell population, with predominantly CD3+ CD4+ T cells intermixed with CD20-positive B cells and polyclonal plasma cells. Most commonly, lupus mastitis is seen in patients with a previous diagnosis of systemic or discoid lupus; however, it can also be the initial presentation of lupus in some patients. We report on 2 cases of lupus mastitis where the clinical impression was to rule out malignancy and review the literature to highlight the key clinicopathologic features.

Acute inflammatory demyelinating polyneuropathy as the initial presentation of lupus

A 28-year-old woman presented at the emergency department (ED) with acute bilateral leg weakness and lost the ability to walk 1 day after noticing bilateral leg numbness and intermittent pleuritic pain for 3 weeks. Physical examination revealed bilateral decreased muscle strength with score 4 symmetrically in the legs and decreased deep tendon reflex. Laboratory tests indicated lymphopenia but no other grossly remarkable findings. Cerebrospinal fluid analysis demonstrated albuminocytologic dissociation. Electrophysiologic survey also indicated sensory-motor demyelinating polyneuropathy. The clinical picture was compatible with acute inflammatory demyelinating polyneuropathy (AIDP), the most prevalent form of Guillain-Barré syndrome (GBS). Plasma exchange was immediately arranged. After serial examination, pleuritic pain, persistent lymphopenia, positive immunologic findings of anti-DNA, and antinuclear antibodies led to a diagnosis of systemic lupus erythematosus (SLE). Prednisolone was added along with plasma exchange. The patient was able to walk after 2 weeks of therapy. Acute inflammatory demyelinating polyneuropathy presenting as the initial manifestation of SLE is rather rare. The precise mechanism of SLE-related AIDP remains unclear but is probably immune related. Although steroids are not recommended in the management of AIDP or GBS, patients with SLE-related AIDP may benefit from steroid therapy. This case highlights that early initiation of evaluation for SLE by ED physicians may facilitate correct diagnosis and better outcomes in patients presenting with GBS or AIDP in the ED.

Mielitis transversa aguda en siete pacientes con lupus eritematoso sistémico

Although the association between transverse myelitis and systemic lupus erythematosus is rather infrequent, it is important to take this form of clinical presentation into account because it is a serious complication, which can potentially be treated but even when dealt with in the early stages does not always have a good prognosis. We conducted a retrospective review over the past 13 years of the cases that have been admitted to our specific centre for the treatment of spinal cord injuries that were diagnosed as suffering from myelitis associated with disseminated lupus erythematosus. Demographic and clinical data, together with data about acute phase and maintenance treatments, as well as the patients' progress and sequelae are described. The case reports of seven patients, all of whom were young females, are studied. In two cases, myelitis was the initial presentation of lupus. The main disorder was at the dorsal, followed by the cervical, levels. Only two patients had a favourable long-term progression from the neurological point of view (both managed to walk) despite acute treatment with high doses of intravenous corticoids, and regardless of the fact that cyclophosphamide was later used. Myelitis associated to lupus is a rare manifestation but, owing to its important functional repercussions, it must be taken into account when faced with an acute clinical picture involving the spinal cord; this is particularly the case when it occurs in young females, with or without a previous diagnosis of autoimmune disease.