Raised Sedimentation Rate Research Articles

Review: Diagnosis and treatment of cerebral vasculitis

Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved.

UK Myeloma Forum (UKMF) and Nordic Myeloma Study Group (NMSG): guidelines for the investigation of newly detected M‐proteins and the management of monoclonal gammopathy of undetermined significance (MGUS)

Avon Haematology Unit, Bristol Haematology and Oncology Centre, Bristol, Department of Haematology, St Helier Hospital, Carshalton, Surrey, UK, Department of Haematology, Sahlgrenska University Hospital, Gothenburg, Sweden, Department of Medicine, Malmo University Hospital, Malmo, Sweden, Division of Immunity and Infection, University of Birmingham, Birmingham, Department of Clinical Biochemistry, Frenchay Hospital, Bristol, Department Haematology, University College, London, Glasgow Western Infirmary, Glasgow, UK, Department of Haematology, NTNU/St Olavs Hospital, Trondheim, Department of Haematology, Ulleval University Hospital, Oslo, Norway, Department of Haematology Aalborg Hospital, Aarhus University Hospital, Aalborg, Denmark, and Myeloma UK.

Sjögren Syndrome or Sjögren Disease? The Histological and Immunological Bias Caused by the 2002 Criteria

The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p < 0.001) and had a lower frequency of parotid enlargement (p = 0.002), fever (p = 0.041), arthritis (p = 0.041), vasculitis (p = 0.050), peripheral neuropathy (p = 0.002), cranial nerve involvement (p = 0.015), raised erythrocyte sedimentation rate ( ESR) levels (p < 0.001), anemia (p < 0.001), leukopenia (p = 0.037), hypergammaglobulinemia (p < 0.001), positive rheumatoid factor ( RF; p = 0.002), and cryoglobulinemia (p = 0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies , complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren "disease." In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren "syndrome" seems more adequate.

Managing children with acute non‐traumatic limp: the utility of clinical findings, laboratory inflammatory markers and X‐rays

To examine the utility of clinical findings, laboratory markers and X-ray radiographs (X-ray) in the assessment of children presenting with an acute non-traumatic limp. A retrospective review of all children who received hip X-rays over a 2 year period in the Children's Emergency Department, Starship Children's Hospital, Auckland, New Zealand. Children were identified from the radiology database and clinical notes reviewed. Children aged 0-12 years old were included if the limp was acute (less than 2 weeks of duration) with no history of trauma. X-rays were reported by a consultant paediatric radiologist. Univariate and multivariate analysis was performed to determine predictors of osteomyelitis and septic arthritis. Receiver operator curves were used to assess the optimum cut-off points for C reactive protein (CRP), erythrocyte sedimentation rate (ESR) and white cell count (WCC). A total of 350 patients were enrolled. There were 21 (6%) abnormal X-rays . Fever, non-weight bearing, raised white cell count, raised erythrocyte sedimentation rate and raised CRP were all associated with increased risk of septic hip or osteomyelitis. The optimum inflammatory marker cut-off was a CRP of 12 with a sensitivity of 87% and specificity of 91%. In acute non-traumatic limp, X-rays of the hips diagnose slipped upper femoral epiphysis, as such they should be routinely used from the age of 9 years upwards. Below this age they are of little value. Inflammatory markers have utility in risk-stratifying children and selecting a group in whom to proceed with definitive tests to exclude osteomyelitis or septic hip. Children with a short history and minimal symptoms can be managed with appropriate follow up and no investigations.

An Unusual Post Gastrectomy Complication

Question: A 62-year-old man presented with repeated episodes of epigastric pain arising about 2 hours postprandially, and radiating to the back. There were associated symptoms of nausea, vomiting, perspiration, and pallor. These symptoms worsened gradually over a few months and were accompanied by a weight loss of 12 kg. The patient underwent a distal gastrectomy (Billroth II) procedure for peptic ulcer disease almost 20 years earlier. Physical examination of the abdomen showed epigastric tenderness, but was otherwise normal. Laboratory analysis showed a raised sedimentation rate (49 mm/H during the first hour, 77 mm/H during the second hour), low hemoglobin (12.9 g/dL), normal leukocyte count (5,470/L), and platelet count (146,000/L). Amylase and lipase were normal (79 U/L and 15 U/L, respectively). His C-reactive protein level was slightly elevated (0.9 mg/dL). Gastroscopy and abdominal ultrasound were normal, but computed tomographic (CT) angiography showed a vascular anomaly (Figures A and B, arrows). What is the diagnosis?

Ewing's sarcoma of the ilium mimicking inflammatory arthritis of the hip: a case report

IntroductionEwing’s sarcoma is second most common primary malignant bone neoplasm in children and adolescents. We report a case of Ewing’s sarcoma of ilium in an 18-year-old female mimicking clinically and radiologically inflammatory arthritis of hip joint, a rare entity not mentioned in literature.Case presentationAn 18-year-old girl presented with pain hip, limp and fever off and on. Patient had restricted range of motion, raised erythrocyte sedimentation rate, leucocytosis and radiograph showed reduced joint space. Magnetic resonance imaging pelvis revealed an altered marrow signal of acetabulum with a large soft tissue component. Histopathology revealed a malignant round cell tumor consistent with Ewing’s sarcoma.ConclusionClassical clinical and radiological presentation of Ewing’s sarcoma of ilium may not be the rule. One should be highly suspicious of the disease even if there is no direct pointer to the disease as was encountered in our case.

IMMUNOTHERAPY-REVERSED COMPULSIVE, MONOAMINERGIC, CIRCADIAN RHYTHM DISORDER IN MORVAN SYNDROME

Antibodies to voltage-gated potassium channels (VGKC-Abs) have been recognized in neuromyotonia, limbic encephalitis (LE), and Morvan syndrome (MoS),1,2 a rare condition with insomnia, peripheral, and central and autonomic nervous system involvement. Clinical progression in MoS is variable, but potentially fatal. We describe a patient with VGKC-Abs-positive MoS who showed prominent compulsive behaviors, basal ganglia hypermetabolism, increased catecholamine and serotonin secretion, epileptic seizures, and cardiac and endocrine circadian rhythm suppression. A striking response to immunotherapy paralleled a marked reduction in VGKC-Abs over 15 months. ### Case report. A 64-year-old man with idiopathic pulmonary fibrosis and polyarthrosis developed cramps, lower extremity pain, and sensory loss. Three years later, his sleep was disturbed by chewing, manual stereotypies, and sleep-talking. After an episode of tonic-clonic seizures, EEG revealed a temporal epileptic focus. He developed paroxysmal confusional episodes, with gesturing and irregular breathing, that were unresponsive to multiple anticonvulsants. He became irritable and hypomanic with overwhelming compulsive shopping and stealing. Urinary frequency, anosmia, impotence, and increased appetite appeared. Examination showed bilateral postural tremor, multifocal myoclonus, lower extremity fasciculations, hypoesthesia, hyporeflexia, and mild proximal muscle atrophy. Laboratory investigations, including autoimmune and thyroid profile, were normal except for increased leukocytes, a raised erythrocyte sedimentation rate and creatine phosphokinase, mild hyponatremia, and antinuclear antibodies 1:160. CSF was negative for infections and 14.3.3 protein, but showed slightly increased …

Bilateral thalamic enlargement in an acutely ill infant

A 2-month-old male baby from the eastern part of India presented with fever for 3 days, seizures for 1 day and loss of consciousness for 6 h. Contact with another sick child or with tuberculosis was denied. The baby was febrile, had tachycardia with tachypnea and features of circulatory failure. Blood examination revealed normal blood glucose with leukocytosis and raised erythrocyte sedimentation rate. He had elevated blood urea but serum electrolytes were normal.

Bilateral thalamic enlargement in an acutely ill infant

A. Japanese encephalitis. Bilateral thalamic enlargement in an acutely ill infantJournal of Clinical NeuroscienceVol. 15Issue 12PreviewA 2-month-old male baby from the eastern part of India presented with fever for 3 days, seizures for 1 day and loss of consciousness for 6 h. Contact with another sick child or with tuberculosis was denied. The baby was febrile, had tachycardia with tachypnea and features of circulatory failure. Blood examination revealed normal blood glucose with leukocytosis and raised erythrocyte sedimentation rate. He had elevated blood urea but serum electrolytes were normal. Full-Text PDF

Tuberculous meningitis presenting with unusual clinical features in Nigerians: Two case reports

BackgroundTuberculous meningitis is common in developing countries and accounts for about 7.8% to 14% of all cases of tuberculosis in Nigeria.Case presentationCase 1 was a 17-year-old woman who presented with a 3-week history of weakness of the right upper and lower limbs, a 6-hour history of inability to speak and irrational behaviour. She had no remarkable past medical history. Physical examination revealed pyrexia (temperature of 38.2°C) and altered level of consciousness (Glasgow coma score = 7/15). The signs of meningeal irritation were present and she had anisocoria and right spastic hemiparesis. Other aspects of physical examination were normal. Laboratory investigations showed an elevated erythrocyte sedimentation rate, normal cerebrospinal fluid protein and reduced glucose. The brain computed tomography scan showed features in keeping with obstructive hydrocephalus and she was immediately commenced on antituberculous drugs, intravenous steroids and mannitol. She made a remarkable clinical recovery and was discharged home 6 weeks after admission. Case 2 was a 40-year-old man who presented with a 6-week history of headache and fever and a 2-week history of alteration in level of consciousness. There was no history of neck pain and/or stiffness, nausea or vomiting. He had no other remarkable past medical history. He had been placed on various intravenous antibiotics in private hospitals before presentation, with no clinical improvement. Physical examination showed a young man in a coma (Glasgow coma score = 4/15) and febrile (temperature of 38.5°C) with signs of meningeal irritation. The brain stem reflexes were impaired and he had spastic quadriparesis. Further physical examination was essentially normal. The cerebrospinal fluid analysis showed features in keeping with meningeal inflammation and he had a raised erythrocyte sedimentation rate. The brain computed tomography scan showed features in keeping with obstructive hydrocephalus. He was placed on antituberculous drugs and intravenous steroids but despite this his clinical condition deteriorated and he died on the sixth day after admission.ConclusionLate presentation of tuberculous meningitis is not rare in Nigerians and we report two cases of tuberculous meningitis that presented late to our health care facility. This report is intended to make clinicians aware of the unusual clinical presentations of tuberculous meningitis.

Primary septic arthritis of the acromio-clavicular joint: case report and review of literature

Primary septic arthritis of the Acromio clavicular joint is an uncommon disorder and is rarely seen even in an immunocompromised individual. We report a case of primary septic arthritis of the acromio-clavicular (A-C) joint caused by Staphylococcus aureus without any predisposing factors. The patient was admitted with left shoulder pain, restricted movements and fever. Laboratory parameters showed high C-reactive protein, raised erythrocyte sedimentation rate and leucocytosis. Blood cultures were positive for Staphylococcus-aureus. Magnetic resonance imaging (MRI) using Gadolinium enhancement revealed marked effusion in the A-C joint. Aspiration from the A-C joint revealed a heavy growth of Staphylococcus-aureus. The patient was successfully treated with 8 weeks of appropriate antibiotics with complete resolution of infection and return to full function.

Spinal infection caused by ESBL-producing Klebsiella pneumoniae treated with Temocillin

A case of cervical epidural abscess in a 63-year-old woman with a complex gastrointestinal surgical history is reported. The presenting symptom was neck pain, and blood analysis demonstrated raised erythrocyte sedimentation rate and C-reactive protein. MR imaging of the cervical spine revealed cervical discitis, osteomyelitis, and epidural collection. An ESBL-producing strain of Klebsiella pneumoniae, isolated from a previous episode of bacteraemia, was the causative organism. Surgical treatment was not possible and initial carbapenem therapy was aborted due to development of neutropaenia, and treatment continued with an extended course of temocillin. After 12 weeks of antibiotics the patient was pain-free with radiological improvement and resolution of blood markers of infection.

Longitudinal study of 16 patients with Takayasu’s arteritis: clinical features and therapeutic management

Takayasu's arteritis (TA) is a rare vasculitis that affects the aorta and its primary branches. Heterogeneous clinical manifestations have been described in different geographical areas. We aimed to evaluate demographic, clinical, and angiographic features and the outcome in 16 TA patients who were followed in a single center in Serbia. TA was diagnosed according to the 1990 American College of Rheumatology criteria with median delay of 2 years. The majority of patients (81%) experienced one or more disease relapses and long-term remission was achieved in 94%. Five-year survival rate was 94%. Common systemic manifestations at disease onset included fever (69%), arthralgia (25%), weight loss (19%) and vascular symptoms, arm claudication (100%), headaches (69%), dizziness (56%), and hypertension (37%). All patients had multiple arterial bruits with diminished/absent left arm pulse in 88%. The raised erythrocyte sedimentation rate and C-reactive protein levels were found in 63% and 80%, respectively. Angiography revealed stenotic or occlusive sites of multiple arterial involvement. Stenosis of the left subclavian artery was the most frequent lesion (88%). The angiographic classification was as follows, 50% type I, 19% type IIa, and 31% type V arteritis, suggesting relatively favorable vascular lesions and infrequent serious complications. Glucocorticoids were the main initial therapy for active disease; however, treatment with additional immunosuppressive agents was required in two-thirds of the patients. Seven patients (44%) underwent one or multiple surgical interventions. The main indications for surgery were cerebral hypoperfusion, severe limb claudication, and gangrenous infection. The demographic and angiographic disease patterns in our cohort were similar to American, Italian, Japanese, and Korean cohorts in terms of frequent involvement of the branches of aortic arch and differed from cohorts from other Asian countries in which abdominal aorta and renal artery involvement prevailed.

A reassessment of diagnostic criteria and treatment of idiopathic urticarial vasculitis: a retrospective study of 47 patients

Urticarial vasculitis (UV) is an uncommon type of chronic urticaria (CU), which exhibits leucocytoclastic vasculitis. Painful and long-lasting (> 24 h) weals associated with purpura or bruising are considered indicative of UV. It is often responsive to oral corticosteroids and poorly to oral antihistamines. Hypocomplementaemia and systemic involvement are also commonly reported. To diagnose patients with UV histologically and then compare their clinical features and response to various treatment regimens. Biopsies were taken from 312 subjects with CU unresponsive to oral antihistamines; of these, 47 were histologically diagnosed as having UV. Biopsies were taken irrespective of the clinical features of weal eruption. Other diseases known to be associated with small-vessel vasculitis had previously been excluded. Results. Individual weals lasted < 24 h in 57.4% of patients, and pain or tenderness was reported only by 8.6%. Extracutaneous features were present in 81%, hypocomplementaemia in 11% and abnormalities of other laboratory parameters (i.e. raised erythrocyte sedimentation rate, microscopic haematuria) in 76.6%. Hydroxyzine was effective in only one patient. Both oral corticosteroids and cinnarizine were effective in a high percentage of the patients. This diagnostic approach allowed us to identify a large group (47 patients) with UV. Most did not present the clinical (prolonged duration of weals and bruising) and laboratory features that have previously been described as characteristic of UV. Cinnarizine was found to be a valuable treatment option.

A patient with hyper-IgD syndrome responding to simvastatin treatment

The hyper-IgD syndrome (HIDS) is an autosomal recessively inherited auto-inflammatory syndrome, caused by deficient enzyme activity of mevalonate kinase (MVK), an enzyme in the isoprenoid pathway. Patients present with a long history of recurrent fever attacks, accompanied by abdominal pain, skin lesions, lymphadenopathy and arthralgia. The attacks last about 3–7 days followed by a spontaneous remission, only to recur after a symptom-free period of 4–6 weeks. Factors known to precipitate an attack are infection, trauma, vaccination, and psychological stress. Laboratory examination at the time of attacks reveals a vigorous acute phase response with leucocytosis, raised sedimentation rate, high serum concentrations of C-reactive protein and increased mevalonic acid urinary levels. Almost all patients have an elevated polyclonal immunoglobulin D. Until now, treatment of HIDS patients is largely supportive and very difficult. Various anti-inflammatory drugs have failed to suppress the fever attacks [ [1] Haas D. Hoffmann G. Mevalonate kinase deficiencies: from mevalonic aciduria to hyperimmunoglobulinemia D syndrome. Orphanet J Rare Dis. 2006 Apr; : 1-13 Google Scholar ].

Rapidly enlarging mediastinal mass in a middle-aged patient with fever

A 47-year-old Indian female patient presented with lowgrade intermittent fever, anorexia and malaise of 4 months’ duration. She had a history of disseminated tuberculosis 2 years previously, for which she was successfully treated with first-line anti-tubercular drugs. On physical examination, she was found to have multiple matted cervical and axillary lymph nodes with mild splenomegaly. Laboratory results showed a raised erythrocyte sedimentation rate. Chest radiograph was normal (Figure 1). CT of the chest revealed multiple confluent rim-enhancing necrotic lymph nodes in the right axilla (Figure 2a). There was no mediastinal or hilar adenopathy and the lungs were clear (Figure 2b). Axillary lymph node biopsy was positive for acid fast bacilli. The patient was started on a modified antitubercular treatment regimen; however, her compliance for the chemotherapeutic drugs was poor and she continued to have constitutional symptoms. 5 months later, her condition worsened and she again presented to us with high-grade fever, anorexia, fatigability

Identification of Cowpox Infection in a 13-year-old Danish Boy

developed into a pustule. He initially had systemic symptoms with high-grade fever up to 39.5oC and flu-like symptoms. At this stage he sought medical attention by an otologist who initially prescribed dicloxacillin and, when this had no effect, erythromycin, as bacterial infection was suspected. The fever abated in 4–5 days, but the neck lesion continued to enlarge and ulcerated during the second week. At the time of referral the patient presented with an indurated, indolent ulcer, 3 cm in diameter, with a hard, black eschar surrounded by erythema and local lymphadenopathy. He was treated with oral antibiotics until the viral aetiology was established and local antiseptic treatment was continued to prevent secondary infection. The lymphadenopathy gradually subsided and the ulcer healed in 6–8 weeks with some scarring. Laboratory investigations revealed a slightly raised erythrocyte sedimentation rate, while leukocyte counts, C-reactive protein and liver function tests were normal. Cutaneous anthrax and impetigo were excluded by negative swab culture from the ulcer. Swabs from the lesion were negative, as were serological tests for Bartonella henselae and Francisella tularensensis. Biopsy samples obtained for Mycobacterium tuberculosis culture were also negative. Two 4-mm punch biopsies were taken from the rim of the ulcer. Light microscopy of haematoxylin and eosin (H&E)-stained sections showed non-specific inflammatory changes in the epidermis and dermis. No cytoplasmic inclusions were seen. The swab sample was analysed initially using MagNA Pure extraction method (Total NA Isolation Kit, no. 3 038 505, Roche, Mannheim, Germany). To improve sensitivity, an additional extraction was performed subsequently, using increased sample volumes (1000 µl) combined with decreased elution volumes (50 µl). Ulcer biopsy material was split and prepared for electron microscopy and cultivation of mycobacteria, for which the latter was treated with BLL™ MycoPrep™ (BD Diagnostics, Franklin Lakes, NJ, USA) according to the manufacturer’s instructions. This material was extracted using the modified protocol.

Bilateral periocular psoriasis: an initial manifestation of acute generalized pustular psoriasis with coexistent Sjögren's syndrome

A 21-year-old febrile lady presented to the emergency service with severe lid oedema, conjunctivitis, dry mouth and abdominal skin rash. Over 5 days, she developed silvery scales and pustules on the lids, and generalized pustules on an erythematous base. Multiple focal sterile corneal infiltrates were seen. Haematological investigations and a skin biopsy were done as the consulting dermatologist suspected acute generalized pustular psoriasis. In addition, secondary Sjögren's syndrome was diagnosed since she had keratoconjunctivitis sicca, xerostomia, raised erythrocyte sedimentation rate and positive antinuclear antibodies. The presence of microabscesses in the epidermis on skin biopsy confirmed the diagnosis of pustular psoriasis. With oral methotrexate 7.5 mg weekly and topical corticosteroids, the acute condition gradually resolved; however, the keratoconjunctivitis sicca is persisting. Secondary Sjögren's syndrome associated with acute generalized pustular psoriasis and ocular psoriasis is extremely rare. Awareness of the ocular and dermatological features of these two conditions would result in earlier diagnosis and institution of appropriate treatment.

Inner Table Sequestrum

Inner Table Sequestrum

Leptospirosis as a cause of fever in pregnancy

Leptospirosis is a zoonosis. Rodents especially rats are the reservoir and humans are not commonly infected. Leptospirosis should be included in a differential diagnosis along with malaria enteric fever viral hepatitis and dengue in a patient with febrile illness associated with headache and muscle pain. More than 90% of symptomatic people have mild disease: fever chills headache myalgia nausea and vomiting. On examination fever conjunctival suffusion lymphadenopathy pharyngeal injection rash and hepatosplenomegaly may be present. In severe form it is characterized by jaundice renal dysfunction and hemorrhagic diasthesis with a mortality of 5% to 15%. Proteinuria raised erythrocyte sedimentation rate and leukocytosis with neutrophilia are the most indicative clinical laboratory findings for early diagnosis. Serum aspartate aminotransferase alanine aminotransferase and lactic dehydrogenase levels are slightly elevated even in patients with severe jaundice which differentiates it from viral hepatitis. A definite diagnosis is made on either isolation of the organism from the patients urine blood or cerebrospinal fluid or by serologic specific test. In severe cases a pulmonary radiograph may show a patchy alveolar pattern. (excerpt)