Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 14-year-old girl was admitted to the hospital because of persistent throat pain, fever, fatigue, 25 pound weight loss, and leukopenia. On physical examination she was thin, ill-appearing, and had necrotic papules on the face and palpable cervical lymph nodes. Presumptive differential diagnosis included occult malignancy and infection. Numerous investigative procedures failed to elucidate a source. Vasculitis was eventually appreciated after repeat skin biopsy. Numerous serologic studies were performed and were notable for a very low level of the second component of complement without direct evidence of lupus erythematosus (LE) or other autoimmune conditions. A diagnosis of C2 deficiency-associated vasculitis was made. She was treated with high-dose prednisone and cyclophosphamide with resolution of her symptoms. Two years later she returned with marked malar erythema. Antinuclear and Smith antibodies were then detected and a diagnosis of LE was made. She was treated with hydroxychloroquine and sun-avoidance measures with clearance of the malar rash.
 ...
PMID:Necrotic facial papules in an adolescent: C2 deficiency with eventual development of lupus erythematosus. 1286 53

Infliximab is a tumour necrosis factor (TNF)-alpha antagonist that has revolutionised the treatment of Crohn's disease and rheumatoid arthritis. However, infliximab therapy can be complicated by a variety of adverse reactions. Acute infusion reactions occur during or shortly after infusion and typically consist of fever, chills, nausea, dyspnoea and headaches. Delayed reactions, characterised by myalgias, arthralgias, fever, rash, pruritus, facial, hand or lip oedema, dysphagia, urticaria, sore throat and headache may occur 3-12 days after infusion. Although the mechanisms of these reactions are not yet clearly defined, emerging evidence indicates that these reactions may be associated with the immune response against infliximab and the development of antibodies to infliximab.A number of studies have identified protective factors that may minimise adverse reactions, presumably related to the immune response against infliximab. Factors that may be protective by helping to establish immune tolerance for the foreign infliximab protein include concomitant administration of immunomodulators or corticosteroids, starting infliximab therapy with a 0, 2, 6-week induction regimen, maintenance dose administration with infusions every 8 weeks or less, and avoiding long periods between infusions. Infliximab therapy also may have other immunological consequences. There is evidence that infliximab may impede the appropriate immune response to a number of pathogens, prohibiting its use in patients with active infections. In addition, patients should be screened and appropriately treated for tuberculosis before initiating infliximab therapy. The development of autoantibodies, such as antinuclear antibody or anti-ds-DNA, has also been described with infliximab therapy, although the development of clinical lupus-like syndrome is rare. While there is a theoretical risk of increased rate of malignancies due to antagonism of TNFalpha, to date there is no clear evidence of such an effect. In addition, cardiac and neurological adverse events associated with infliximab therapy have been described. The mechanism for these adverse events is unclear. In summary, infliximab therapy can be an effective treatment for Crohn's disease; however, a number of immunological consequences and adverse events may complicate the infusion of this agent. Appropriate prophylaxis and therapy of these adverse reactions will allow infliximab to be used safely in the vast majority of patients.
 ...
PMID:Managing immunogenic responses to infliximab: treatment implications for patients with Crohn's disease. 1530 61

A 42-year-old woman with a 24-year history of systemic lupus erythematous and lupus nephritis for 8 years who had been receiving regular hemodialysis for 4 years for nonoligoric end-stage renal disease (ESRD) ingested about 100 mL of 40.8% chlorpyrifos in a suicide attempt. On admission to our emergency department, she was drowsy. Gastric lavage, activated charcoal, pralidoxime (PAM), and atropine were administered 4 h later. Her consciousness level improved gradually with treatment, which included hemodialysis. However, on the second hospital day, intermittent fever to 38.4 degrees C, sore throat, and trismus were noted. About 45 h after chlorpyrifos ingestion, the patient developed profound motor paralysis followed by respiratory arrest, consistent with the diagnosis of intermediate syndrome (IMS), even with adequate atropine and PAM. Chorealike involuntary movements of her upper limbs were noticed on the fifth day. Intermittent tonic-clonic seizures, each attack lasting for 3 to 5 min, appeared on the 13th day, which responded well to intravenous diazepam and phenytoin. She was discharged on the 18th day. This case suggests that patients with ESRD suffering chlorpyrifos intoxication are at risk of IMS. Prompt endotracheal intubation, intensive care, and hemodialysis are necessary for life support.
 ...
PMID:Intermediate syndrome after organophosphate intoxication in patient with end-stage renal disease. 1653 82

We report a case of acquired thrombotic thrombocytopenic purpura (TTP) in a 34-year old patient with a prior diagnosis of systemic lupus erythematosis (SLE) who was recently started on hydroxychloroquine. Presenting symptoms included fevers, sore throat and productive cough with progressive weakness, dyspnea on exertion, hemoptysis and dark urine. Initial laboratory abnormalities were consistent with an acute microangiopathic hemolytic anemia and severe thrombocytopenia. At the time of admission, the patient's lupus was highly active as evident by his high SLE Disease Activity Index (SLEDAI) score. He was later also found to have severely reduced ADAMTS-13 levels and a positive antibody assay. This case highlights the occasional difficulty in pinpointing the exact etiology of TTP as well as establishes a possible novel drug association between hydroxychloroquine and TTP development.
 ...
PMID:Acquired thrombotic thrombocytopenic purpura: puzzles, curiosities and conundrums. 2081 10

Ocular involvement sometimes occurs with systemic lupus erythematosus (SLE) but enophthalmos with SLE is rare. We report a case of enophthalmos with SLE. A 25-year-old male was admitted for two weeks of fever, sore throat, arthralgia, chest pain and right arm weakness with pain. We diagnosed him with SLE with malar rash, arthritis, pleural effusion, proteinuria, leukopenia, positive antinuclear antibody, anti-dsDNA, and lupus anticoagulant. The patient was prescribed high-dose prednisolone and hydroxychloroquine 400 mg. One week after discharge, he complained about a sensation of a sunken right eye. CT showed right enophthalmos, a post-inflammatory change and chronic inflammation. Proteinuria increased to 3.8 g/day after the patient stopped taking prednisolone. Cyclophosphamide therapy was administered for three months without improvement. We decided to restart prednisolone and change cyclophosphamide to mycophenolate mofetil. Proteinuria decreased but enophthalmos remains as of this reporting.
Lupus 2016 Jan
PMID:Acquired enophthalmos with systemic lupus erythematosus. 2630 41