Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies.

OBJECTIVES: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. METHODS: We conducted a multicentre, international, retrospective cohort study. RESULTS: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. CONCLUSIONS: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Increase in body temperature during migraine attacks.

INTRODUCTION: Intermittent fever has been occasionally related to migraine, either as a migraine equivalent or as a migraine accompaniment. We present a case of recurrent increase in body temperature consistently associated with migraine headaches. METHODS: A 15-year-old girl reported a 3-year lasting history of migraine without aura, with a feeling of warmth occurring in each episode. Ancillary tests did not show any evidence of secondary headaches or any systemic disease. A 2-month headache diary was obtained, with daily records of headache intensity (0, no headache; 1, mild pain; 2, moderate pain; 3, severe pain) and simultaneous measurements of axillary temperature. Both parameters were registered in the evening, at 6:00 pm every day. The distribution of headache intensity and body temperature as well as the relationship between both variables over time were analyzed with nonparametric tests. RESULTS: The number of days without pain was 28 (45.2%); a mild headache was present on 13 days (21%), a moderate headache on 15 days (24.2%), and a severe headache on 6 days (9.7%). Headache days were associated with higher body temperature than headache-free days (median values: 37.3°C vs 36.6°C; Mann-Whitney U-test, P < 0.001). Moreover, a positive correlation was found between headache intensity and body temperature (Spearman's rho coefficient: 0.83, P < 0.001). CONCLUSIONS: Recurrent increase in body temperature may be another manifestation of the complex clinical spectrum of migraine. This symptom is probably related to hypothalamic involvement.