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 72-year-old woman presented with multiple sites of soft tissue bleeding and a left ulnar neuropathy due to a compartment syndrome. There were also clinical signs of hypothyroidism. Factor VIII:C (FVIII) levels were 6% and a FVIII inhibitor with a titre of 9 Bethesda units was detected. She had biochemical evidence of hypothyroidism and high titres of antithyroid antibodies were detected. Two episodes of life- or limb-threatening haemorrhage were successfully treated with FEIBA. The inhibitor disappeared following 2 months of immunosuppression with oral cyclophosphamide and corticosteroids. She then developed autoimmune haemolytic anaemia, which responded to further immunosuppression with oral prednisolone. The association between acquired haemophilia and systemic autoimmune disorders such as systemic lupus erythematosis is well recognized, but our description of an association with organ-specific autoimmunity is more unusual.
 ...
PMID:Acquired haemophilia in association with organ-specific autoimmune disease. 1155 45

Compartment Syndrome (CS) is a disease that has 2 etiologies, that of acute events and that of chronic. It occurs when the pressure in a fascia-encased compartment exceeds the profusion pressure in tissue. The end result, when left untreated, is muscle and nerve ischemia that can cause significant morbidity. Nerve paralysis, muscle necrosis and fibrosis and, when occurring in an extremity, loss of the limb are some of the potential outcomes of missed diagnosis. This case series involves 2 cases of CS that where caused by vasculitis with etiologies of human immunodeficiency virus and systemic lupus erythematosis. Autoimmune vasculitis has many systemic and local manifestations, but to our knowledge CS has not been described as one of its sequelae. The following is literature review and presentation of these 2 cases.
 ...
PMID:Acquired immune deficiency syndrome and systemic lupus erythematosis: potential causes of surgical emergencies of the hand. 1858 Jan 47

We report a patient with Down syndrome, under treatment with carbamazepine, levopromazine and clonazepam. After urinary infection he developed glans necrosis requiring excision of prepuce. Six hours post surgery he presented right-hand ischemia followed by arterial and venous thrombosis of the right thoracic extremity. Later, he progressed to a compartment syndrome and presented ischemia of toes. All the clinical manifestations developed over a week. Anticardiolipin (aCL) antibodies, lupus anticoagulant and perinuclear antineutrophil antibodies were positive. Anticoagulant and immunosuppressive treatment were initiated. Owing to the failure of both treatments, the patient underwent amputation of right hand and a toe. Histopathology revealed recent and old thrombosis of medium- and small-sized vessels without vasculitis. Diagnosis of catastrophic antiphospholipid syndrome (CAPS) was made. At present, the patient continues on oral anticoagulants, IgG aCL remains positive, and no further episodes of thromboses have been observed after 4 years of follow-up. To our knowledge, this is the first case of CAPS in a patient with Down syndrome.
Lupus 2009 Oct
PMID:Catastrophic antiphospholipid syndrome in a patient with Down syndrome. 1976 87

This article presents a case of a patient with popliteal artery occlusion following anterior and posterior instrumented fusion of the lumbar spine. No previous study has reported acute anterior tibial compartment syndrome due to popliteal artery occlusion and restricted venous return following spine surgery. A 53-year old female, with a twice failed fusion of L5-S1, underwent L3-S1 anterior interbody and posterior L3-S1 instrumented fusion. Due to postoperative continuous analgesia, the patient was sleepy and confused on postoperative day 1. On the postoperative day 2, the right calf and anterolateral tibia manifested clinical signs of compartment syndrome and both thighs exhibited pressure ecchymoses from the antiembolism stockings. Fasciotomies of the right tibial compartments were undertaken and necrosis of the anterior compartment muscles was found. Intraoperative arteriogram revealed occlusion of the right popliteal artery and thrombectomy was performed. Lupus anticoagulant was found to be responsible for patient's coagulopathy. During postoperative year 1, the patient still had weakness and recurrent edema of the right foot. Unrecognized limb ischemia and possibly restricted venous return were the causes of the compartment syndrome. Surgeons should be aware of this devastating complication of spine surgery.
 ...
PMID:Acute tibial compartment syndrome following spine surgery. 2080 63

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that can lead to damage to several vital organs. Antiphospholipid syndrome (APS), manifesting as vascular thromboembolic events and morbidities of pregnancy in the presence of antiphospholipid antibodies (aPL), has been described in patients with SLE. Catastrophic antiphospholipid syndrome (CAPS), in contradistinction to APS, is defined as three or more organs affected by thrombotic microangiopathy in patients demonstrating aPL and can result in mortality up to 50%. We describe a unique SLE patient who was diagnosed with recurrent APS presented with axillary venous thrombosis and subsequent superficial edema and compartment syndrome. The CAPS followed and revealed thromboses over liver, spleen, and acute pancreatitis. The spontaneous hemorrhage of left fourth intercostal artery (ICA) and left axillary artery occured at the same time without vasculitis or severe trauma. Though emergency transcatheter arterial embolization (TAE) of the left fourth ICA was successfully accomplished by the radiologist. The repeated computed tomography angiogram of chest demonstrated remission of ruptured ICA. Nevertheless, the patient died of diffuse alveolar hemorrhage and respiratory failure and shock. Both disseminated intravascular coagulation (DIC) and CAPS share similar characteristics encompassing thrombotic microangiopathy, bleeding, thromboembolism, and multiple organ dysfunction. It is difficult to distinguish between them, especially in cases such as our uremic SLE patient with a calamitous disease progression. The emphasis of treatment for DIC is on platelet and fresh plasma transfusion, in contrast with anti-coagulant for CAPS. To the best of our knowledge, this is the first report describing ICA hemorrhage in an SLE patient without vasculitis or aneurysm. The lupus flare initiated a pathological immunological cascade and resulted in the CAPS and the vascular damage.
 ...
PMID:A spontaneous intercostal artery hemorrhage in systemic lupus erythematosus. 2133 30

Gastrointestinal manifestations of systemic lupus erythematosus (SLE) are common, occurring in about 50% of cases. They are usually mild, in the form of mouth ulcers, nausea, heartburn and mild abdominal pain, but they can be severe in cases of gastrointestinal vasculitis. In this report we describe an unusual combination of SLE complications, namely superior mesenteric artery syndrome (SMAS) and reversible acute obstructive renal failure. This was attributed to raised intra-abdominal pressure and hence intra-abdominal compartment syndrome (IACS) following weight loss secondary to an acute presentation of SLE with gastrointestinal vasculitis.
Lupus 2014 Feb
PMID:Superior mesenteric artery syndrome and intra-abdominal compartment syndrome in systemic lupus erythematosus. 2433 10