Describing and expanding the clinical phenotype of anti-MDA5-associated rapidly progressive interstitial lung disease: case series of nine Canadian patients and literature review.

OBJECTIVE: To describe and expand the phenotype of anti-MDA5-associated rapidly progressive interstitial lung disease (MDA5-RPILD) in Canadian patients. METHOD: All proven cases of MDA5-RPILD hospitalized in the University of Montreal's affiliated centres from 2004 to 2015 were selected for inclusion. RESULTS: Of nine consecutive patients, RPILD was the presenting manifestation in seven, whereas two patients developed RPILD 2 years after the onset of arthritis and of chronic interstitial lung disease. In the case with arthritis, RPILD was probably triggered by initiation of tumour necrosis factor-α-inhibitor therapy. In most patients (89%), RPILD was accompanied by concomitant onset of palmar/lateral finger papules, skin ulcerations, and/or mechanic's hands. All patients experienced profound weight loss over 1-2 months (mean ± SD 10.2 ± 4.8 kg). All had arthralgias and/or arthritis. Six patients were clinically amyopathic; only one patient had creatine kinase (CK) levels > 500 U/L. Initial ferritin and transaminase levels were elevated in 86% and 67% of patients, respectively. The antinuclear antibody (ANA) test was negative for nuclear and cytoplasmic staining; antisynthetase autoantibodies were negative. Three patients died; time from initial symptoms to death ranged from 7 to 15 weeks. All six survivors received mycophenolate mofetil and/or tacrolimus as part of induction and/or maintenance therapy. CONCLUSION: In an inpatient setting, RPILD associated with characteristic skin rashes, profound weight loss, articular symptoms, normal or low CK with elevated ferritin, and absent fluorescence on ANA testing should alert the clinician to the possibility of MDA5-RPILD. T-cell-mediated therapies may play a role in this highly lethal condition.

Single-specificity anti-Ku antibodies in an international cohort of 2140 systemic sclerosis subjects: clinical associations.

Autoantibodies directed against the Ku autoantigen are present in systemic sclerosis (SSc) and have been associated with myositis overlap and interstitial lung disease (ILD). However, there is a paucity of data on the clinical correlates of anti-Ku antibodies in the absence of other SSc-specific antibodies. The aim of this study was to assess the clinical correlates of single-specificity anti-Ku in SSc.An international (Canada, Australia, USA, Mexico) cohort of 2140 SSc subjects was formed, demographic and clinical variables were harmonized, and sera were tested for anti-Ku using a line immunoassay. Associations between single-specificity anti-Ku antibodies (i.e., in isolation of other SSc-specific antibodies) and outcomes of interest, including myositis, ILD, and survival, were investigated.Twenty-four (1.1%) subjects had antibodies against Ku, and 13 (0.6%) had single-specificity anti-Ku antibodies. Subjects with single-specificity anti-Ku antibodies were more likely to have ILD (58% vs 34%), and to have increased creatine kinase levels (>3× normal) at baseline (11% vs 1%) and during follow-up (10% vs 2%). No difference in survival was noted in subjects with and without single-specificity anti-Ku antibodies.This is the largest cohort to date focusing on the prevalence and disease characteristics of single-specificity anti-Ku antibodies in subjects with SSc. These results need to be interpreted with caution in light of the small sample. International collaboration is key to understanding the clinical correlates of uncommon serological profiles in SSc.

[Treatment of severe accidental hypothermia with a simple extracorporeal circuit]. / Traitement des hypothermies accidentelles graves par un circuit extracorporel simple.

A simple method allows rewarming severe accidental hypothermia patients by an extra-corporal veno-venous circuit. Blood is removed from the femoral vein and brought to infusion pump accelerator which sends it through a blood-rewarmer before reinjecting it into the terminal jugular or sub-clavian vein. This allows the heart to be rewarmed preferentially. The blood is heparinized upon entering the circuit and later neutralized with protamine sulfate upon leaving it. Five severely hypothermic patients (average temperature: 26.5 degrees C) were treated using this technique. Rapid improvement was obtained with disappearance of cardiovascular shock within one hour. Rectal temperature increased by 1.84 degrees C.h-1 and became normal in 6 hours. No complications were encountered. Particularly, there were no incidents of shock due to rapid rewarming and no secondary biological disturbances observed imputable to the extra-corporal circuit. It should be emphasized that the equipment used is readily available in most Emergency and Intensive Care Units.

[Truncal anesthesia of the foot at the level of the ankle: an additional reference mark for the approach to the posterior tibial nerve]. / Anesthésie tronculaire du pied au niveau de la cheville: un repère supplémentaire pour l'abord du nerf tibial postérieur.

Nerve trunk blocks at the ankle could be a most interesting technique of regional anaesthesia. Unfortunately the posterior tibial nerve is difficult to locate with the usual recommended anatomical landmarks (the tibialis posterior artery). The use of the flexor hallucis longus tendon as an additional landmark has been tested in 71 patients scheduled for surgery on the foot (emergency trauma surgery, amputations, ingrowing toe-nails, removal of bedsores, verrucas). Seventy per cent were males. Their age ranged from 3 to 92 years (average 62 years), with 26% being less than 50 years old, and 43% more than 70. A block of the subcutaneous nerves, at the level of the ankle, (medial and lateral sural cutaneous, superficial peroneal, saphenous, medial calcaneal nerves) was followed by a block of the posterior tibial and deep peroneal nerves (sub-aponeurotic nerves). The total number of nerves blocked depended on the use of an ankle tourniquet, and the area involved by surgery. For each nerve blocked, 3 to 6 ml of a mixture containing equal parts of 2% lignocaine and 0.5% bupivacaine were used. The maximum doses injected were 4 mg.kg-1 lidocaine and 1 mg.kg-1 bupivacaine. Anaesthesia was obtained in 10 +/- 3 min, lasting from 180 to 240 min. There were 88.7% excellent results (n = 63), with 7% fair (n = 5) and 4.2% bad (n = 3) results. Failure concerned 5 cases of tibial nerve block, often due to landmark difficulties (great toe previously amputated, significant ankle oedema, lack of operator experience) and, in 3 cases, forgetting to block a nerve involved.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pharyngolaryngeal fistula and hypopharyngeal perforation resulting from difficult tracheal intubation]. / Fistule pharyngo-laryngée et perforation hypopharyngienne après une intubation trachéale difficile.

The authors report a case of lesions of the pharyngo-oesophagolaryngeal junction following difficult intubation. On the basis of the small number of cases published in recent years, they attempt to determine those factors which may permit early diagnosis before the development of complications in order that any medical or surgical therapeutic measures applied may be fully effective.