Synovitis Of Elbow Research Articles

Successful Treatment with Emicizumab of Patient with Type 2N Normandy and Severe Hemophilia a

It is unusual to have both Type 2N VWD and severe hemophilia A. Type 2N (Normandy) von Willebrand Disease (VWD) is an uncommon recessive disorder resulting in a defect in the binding site of von Willebrand Factor (VWF) with factor VIII. Factor VIII plasma levels are low due to rapid clearance. Our patient has severe hemophilia A (factor VIII <1%; Inversion 22) with no history of inhibitors and is heterozygous for a known pathogenic mutation (c2560C>T; p. Arg854Trp) Type 2N “Normandy”. He benefited from the use of HEMLIBRA® (Emicizumab-kxwh, Genentech) a humanized bispecific monoclonal antibody with a long half-life independent of VWF interaction.The patient was diagnosed at 11 months old with excessive bleeding from his frenulum and began episodic standard factor VIII. He had 10 bleeds including knees, arms and hamstring before starting prophylaxis at 3 yrs. of age. On ADVATE 50 units/kg/dose twice a week he had nose bleeds, soft tissue bleeds, and joint bleeds. At 11 yrs. of age, the nose bleeds increased daily. VWD testing revealed VWF activity 25%, VWF Antigen 42%, Factor VIII <1% and Normal Multimers with Type 2 Normandy genotype. ALPHANATE® (Anti-hemophiliac factor/von Willebrand factor complex (human), Grifols) prophylaxis was started to address mucosal bleeding however the right elbow synovitis progressed to right elbow contracture and bone cyst on MRI. He returned briefly to ADVATE® 50 units/kg MWF to every other day prophylaxis with continued intermittent bleeding in right elbow joint. With the diagnosis of Type 2N, and the availability of VONVENDI ® (Recombinant von Willebrand factor, Takeda), he was placed on ADYNOVATE® (Antihemophilic Factor Recombinant PEGylated, Takeda) daily and VONVENDI® every 3rd day starting at 14 yrs. old. The combination of ADYNOVATE® every 12-24 hours with VONVENDI® daily to every other day improved his acute bleeding episodes including his nose bleeds (Half-life ADONYVATE®+VONVENDI® 9.25 hr. vs. Half-life ADVATE® 5.5 hr.). The patient continued to participate in high-risk activities such as playing on trampoline which contributed to his injuries but his family felt the treatment was better than previous therapies.After considering the treatment burden of 2 types of factors more than 5 times a week and progression of synovitis in right elbow joint, the patient was started on HEMLIBRA® with weekly subcutaneous injections. Four weeks of loading dose (3 mg/kg/dose) was followed by once per week prophylaxis at standard dosing 1.5 mg/kg weekly. The first bleeding episode on HEMLIBRA® was reported after tooth restoration October 2019. After receiving both ADYNOVATE® and VONVENDI® pre-op, he developed swelling with suspected hematoma at the angle of his jaw about 4 days post-op treated successfully with ADYNOVATE® /VONVENDI®. In April 2020, he had right elbow pain stereotypic of a joint bleeding episode after yard work which was treated successfully with ADYNOVATE® /VONVENDI®. No report of severe nosebleeds.To our knowledge this is the first example of successful use of HEMLIBRA® in a patient with Type 2N VWD and severe hemophilia A. It is unclear why the ALPHANATE® was not clinically effective however the combination of VONVENDI® and ADYNOVATE® provided improved hemostasis with frequent infusions and high cost before the start of HEMLIBRA®. HEMLIBRA once a week has significantly reduced the treatment burden and improved bleeding prevention. The genotype of this patient is unusual however this approach may be successful in other types of VWD that result in significant bleeding phenotype. [Display omitted] DisclosuresBryant: Novo Nordisk: Consultancy, Honoraria; Bristol Myers Squib: Consultancy, Honoraria; Hema Biologics: Consultancy, Honoraria. Carr: Genentech: Speakers Bureau; Bayer: Speakers Bureau; Medexus: Speakers Bureau.

Effectiveness of arthroscopic elbow synovectomy in rheumatoid arthritis patients: Long-term follow-up of clinical and functional outcomes

To determine the long-term clinical and functional results of arthroscopic elbow synovectomy in rheumatoid arthritis patients with refractory elbow synovitis in terms of improvement in pain, function, and active range of motion (AROM) or arc of motion. Fifteen rheumatoid elbows in 13 patients, not responding to DMARD therapy and with radiological changes not more than Larsen grade 3 were taken, who underwent arthroscopic elbow synovectomy. The main outcome measured in forms of Mayo Elbow Performance Scale (MEPS) score, measurement of pain using a Visual Analogue Scale (VAS), radiological angles of elbow, disease activity score (DAS-28), arc of motions (AOM) and complications, which were assessed at follow-up periods of 6 months, 24 months, and 30 months. Statistical analysis was done both qualitatively and quantitatively. Mann-Whitney U test, chi-square test, and Student t test were used as the statistical test for determining significance. In the study group, the improvement was sustained and significant as compared to baseline (VAS 1.28, MEPS 81.07 and mean flexion range 85°) (p value <0.001). No significant complications were encountered postoperatively after elbow synovectomy. The study assesses the long-term results of arthroscopic synovectomy in elbow synovitis secondary to rheumatoid arthritis with significant results favoring arthroscopic synovectomy.

Comment on "Plant thorn synovitis of elbow in children".

We read with interest the article titled “Plant thorn synovitis of elbow in children”1 and based on our clinical experiences2 would like to share some important aspects of these thorn injuries, which have not been highlighted in this paper. Acacia thorn injuries are common in Northern and Central India, where the Acacia Arabica plants are found in abundance especially in villages and on road side. The sharp thorns of these plants can easily penetrate the exposed parts of the extremities, both upper and lower limbs. These injuries are most commonly seen in foot, as in rural India there is a still a trend towards barefoot walking and/or not wearing strong shoes. The senior author had published already the world's largest series of acacia thorn injuries in 1990,2 in which he described foot to be the most common site (14 out of 22 patients). Incidentally, the present paper has failed to include this important landmark publication in their reference. Acacia Arabica thorns contain severely irritating toxins or alkaloids which trigger severe inflammatory response in the soft tissues and also in the bones.3 The authors would also like to bring to the readers' attention the presence of bony lesions in association with acacia thorn injuries. These lesions may be osteolytic, osteoblastic or mixed lesions and can easily be confused with chronic low grade osteomyelitis or benign neoplasms, if adequate history is not taken.2 Although not noticed in any of the cases in the present series, the radiographs should always be assessed for the presence of any lesions. The authors have described the presence of acacia thorns in the synovium, which were identified macroscopically per operatively. We do not have similar experience, as we had noticed that the acacia thorns if broken and entrapped in the synovium may not be easily visible to the naked eye all the times and hence careful microscopic examination of the total excised tissue should be done to look for the thorns in the specimen. Ultrasonagraphy and MRI can also be useful adjuncts for the diagnosis of thorn injuries. We do agree with the authors that an awareness and high index of suspicion about the possibilities of thorn injuries is crucial in reaching to an early diagnosis and hence a careful history about the foreign body prick, like thorns must be elucidated in all these patients.

Plant thorn synovitis of elbow in children.

Four rare cases of plant thorn synovitis of left elbow were admitted at our institution, which were initially misdiagnosed as partially treated septic arthritis of elbow or Juvenile inflammatory arthritis. All of them were of paediatric age group. Symptoms included pain, swelling, and decreased range of motion of affected joint. On examination synovitis was present in all patients. Roentgenograms & Ultrasonography were inconclusive in all patients, Definitive diagnosis was made only after arthrotomy, Thorn fragments (Acacia arabica) were recovered from the hypertrophied synovium & subtotal synovectomy was done and sent for histopathological examination. All patients improved after surgery with mean residual flexion deformity of 12.5±2.86°. Plant thorn induced synovitis of elbow is rare, it must be included in the differential diagnosis of monoarthritis of elbow and a high index of suspicion is needed for retained thorn fragments in elbow joint causing synovitis. Its optimal treatment is arthrotomy, foreign body removal and total/subtotal synovectomy.

An uncommon cause of elbow synovitis in an adult haemophilia patient

We present the case of an adult haemophilia patient that at clinical examination showed intense painless synovitis in his left elbow, with full range of movement, which was unresponsive to 1 month of on-demand haematologic treatment at home. Radiological examination demonstrated a spontaneous displaced fracture of the olecranon secondary to severe osteolysis, due to advanced haemophilic arthropathy. Then, the patient was treated with a posterior plaster splint to immobilize the joint for 3 weeks together with secondary prophylaxis (3000 IU twice a week for 3 months). One month later the patient was better, with full range of movement, and the amount of swelling had improved. Regarding the outcome of the fracture, 6 months later there was no healing of the fracture, and the patient developed a painless nonunion that allowed him to return to his preinjury activities of daily living. As far as we know, this rare combination of problems (unresponsive synovitis, severe haemophilic arthropathy, spontaneous elbow fracture) has not been previously published in the literature.

Inflammatory posterior interosseous nerve palsy in a patient with psoriatic arthropathy

Psoriasis is a chronic, relapsing, inflammatory skin disorder with a strong genetic basis. Five patterns of psoriatic arthritis have been identified: asymmetrical oligoarticular arthritis, symmetrical polyarthritis, distal interphalangeal arthropathy, arthritis mutilans and spondylitis with or without sacroiliitis. Extra-articular disease is uncommon. We report a rare case of an inflammatory posterior interosseus nerve palsy in a patient with known psoriatic arthropathy, where investigation warranted medical treatment over a surgical approach. The commonest cause of posterior interosseus nerve palsy is entrapment at the proximal forearm. Other possible aetiologies include extension of elbow synovitis as described in rheumatoid arthritis, trauma eg. Monteggia fractures, tumours and iatrogenic injuries. We discuss the diagnostic dilemma and the management issues for upper limb surgeons.

Importance of immobilization after intraarticular glucocorticoid treatment for elbow synovitis: A randomized controlled study

To investigate whether better treatment results might be achieved with postinjection rest following intraarticular glucocorticoid treatment for elbow synovitis. Ninety patients with rheumatoid arthritis and elbow synovitis were treated with 20 mg of intraarticular triamcinolone hexacetonide and randomized to either a 48-hour immobilization in a triangular sling (n = 46) or to normal activity without restrictions (n = 44). The primary end point was relapse of synovitis. In addition, pain, function according to a self-assessment questionnaire (the Patient Rated Elbow Evaluation), and range of movement were followed for 6 months. Thirteen relapses occurred in the rest group and 8 in the active group (results not significant). The secondary measures showed no statistically significant differences between the groups. Immobilization procedures do not improve the outcome of intraarticular glucocorticoid therapy for elbow synovitis. In future guidelines for such treatment of the upper extremity, normal activity after the injection should be recommended.

Posterior interosseous nerve syndrome secondary to rheumatoid synovitis

Three additional cases of posterior interosseous-nerve paralysis secondary to rheumatoid arthritis were seen. All were initially diagnosed as extensor tendon ruptures and in one patient the extensor tendons were explored before proper diagnosis was made. The condition is a compression neuritis of the posterior interosseous nerve secondary to elbow synovitis bulging anteriorly against the overlying supinator muscle. Treatment by injection of steroids was successful in one patient; synovectomy cured the condition in the second; and a tendon transfer was used in the third.

Posterior Interosseous-Nerve Syndrome Secondary to Rheumatoid Synovitis

Three additional cases of posterior interosseous-nerve paralysis secondary to rheumatoid arthritis were seen. All were initially diagnosed as extensor tendon ruptures and in one patient the extensor tendons were explored before proper diagnosis was made. The condition is a compression neuritis of the posterior interosseous nerve secondary to elbow synovitis bulging anteriorly against the overlying supinator muscle. Treatment by injection of steroids was successful in one patient; synovectomy cured the condition in the second; and a tendon transfer was used in the third.

Posterior Interosseous Nerve Syndrome Secondary to Rheumatoid Synovitis

Three additional cases of posterior interosseous-nerve paralysis secondary to rheumatoid arthritis were seen. All were initially diagnosed as extensor tendon ruptures and in one patient the extensor tendons were explored before proper diagnosis was made. The condition is a compression neuritis of the posterior interosseous nerve secondary to elbow synovitis bulging anteriorly against the overlying supinator muscle. Treatment by injection of steroids was successful in one patient; synovectomy cured the condition in the second; and a tendon transfer was used in the third.