Clinical profile and treatment outcomes in antisynthetase syndrome: a tertiary centre experience

Objectives The aim was to describe the clinical profile and outcomes in patients with antisynthetase syndrome (ASS) from a tertiary care centre. Methods The clinical data and investigations of all patients classified as ASS by Connors criteria over 5 years were recorded, and they were followed up prospectively. The median (interquartile range) was used for descriptive statistics. Clinical variables between the Jo-1 and non-Jo-1 groups and between patients with and without anti-Ro52 antibodies were compared using the χ2 test. Survival analysis was done using the log rank test. Results The 28 patients (23 females) had a median age of 42.5 (34.8–52.3) years, with a disease duration of 1.75 (0.6–3.8) years at diagnosis, and had a follow-up of 2 (0.25–4.25) years. Seronegative arthritis was seen in 23 of 28 patients. Non-specific interstitial pneumonia was seen in 19 patients with interstitial lung disease (ILD). Antibodies to Jo-1 (n = 17) were more frequent than non-Jo-1 antibodies (n = 11; five anti-PL-12, four anti-PL-7 and two anti-EJ). There was no significant difference in the prevalence of myositis (P = 0.07) or ILD (P = 0.11) between groups. Anti-Ro52 antibodies were more frequently found in the non-Jo-1 group (P = 0.006, ϕ = 0.51). A partial or complete improvement with treatment was seen in three-quarters of the patients. Five patients succumbed to the illness. Better survival was seen in the Jo-1 group (P = 0.05). Conclusion The most typical presenting manifestation of ASS in our cohort was isolated seronegative arthritis. Non-specific interstitial pneumonia was the commonest ILD pattern. Patients with antibodies to Jo-1 had better survival compared with non-Jo-1. The non-Jo-1 aminoacyl-transfer RNA synthetases had a strong association with anti-Ro52 antibodies.

Giant Melon seed Bodies in Painless Shoulder Swelling

Melon seed bodies are a non-specific manifestation of chronic synovial inflammation associated with various arthritides such as tubercular, rheumatoid, and seronegative arthritis. Characteristic magnetic resonance imaging (MRI) appearance of these bodies differentiates them from other differential diagnoses (such as synovial chondromatosis and pigmented villonodular synovitis). Ultrasonography and MRI, in combination with clinical and laboratory details, can suggest the possible etiology. Medical management of the underlying cause, along with arthroscopic removal of pathognomonic bodies, is the preferred treatment.

Comparing the Clinical and Laboratory Features of Remitting Seronegative Symmetrical Synovitis with Pitting Edema and Seronegative Rheumatoid Arthritis

In seronegative arthritis with extremity edema, it is difficult to differentiate between remitting seronegative symmetrical synovitis with pitting edema syndrome (RS3PE) and seronegative rheumatoid arthritis (SNRA). We compared the clinical characteristics of RS3PE and SNRA in patients with and without malignancies. We retrospectively examined patients diagnosed with RS3PE (McCarty criteria) and SNRA at our hospital in 2007–2020. Malignancy was diagnosed within 2 years before or after RS3PE or SNRA diagnosis. Overall, 24 RS3PE and 124 SNRA patients were enrolled. The median ages were 79.5 and 68.5 years, and men comprised 54.2% and 37.1% of RS3PE and SNRA patients, respectively. RS3PE patients had higher inflammation levels (p = 0.004) and more incidences of malignancy (p = 0.034). Matching for age and sex, RS3PE patients had higher inflammation levels (p = 0.021) and more incidences of malignancy (p = 0.005). Overall, odds ratios (ORs) for malignancy were higher for older age (OR 1.06, p = 0.037), male sex (OR 4.34, p = 0.007), RS3PE patients (OR 4.83, p = 0.034), and patients with extremity edema (OR 4.83, p = 0.034). Inflammation levels and associated factors of malignancy were higher in RS3PE patients than in SNRA patients. Patients who are older, male, with extremity edema, or had RS3PE should be screened for malignancies.

Comparing the Clinical and Laboratory Features of Remitting Seronegative Symmetrical Synovitis with Pitting Edema and Seronegative Rheumatoid Arthritis: Stage 1

In seronegative arthritis with extremity edema, the differential diagnosis between remitting seronegative symmetrical synovitis with pitting edema syndrome (RS3PE) and seronegative rheumatoid arthritis (SNRA) is difficult. We compared the clinical characteristics of RS3PE and SNRA and those of such patients with and without malignancies. We retrospectively examined patients diagnosed with RS3PE (McCarty criteria) and SNRA at our hospital in 2007–2020. Malignancy was diagnosed within 2 years before or after RS3PE or SNRA diagnosis. Overall, 24 RS3PE and 124 SNRA patients were enrolled. The mean ages were 79.0 and 66.5 years, and men comprised 54.2% and 37.1% of RS3PE and SNRA patients, respectively. RS3PE patients had higher inflammation levels (p < 0.01) and more incidences of malignancy (p < 0.01). Matching for age and sex, RS3PE patients had higher inflammation levels (p < 0.01) and more incidences of malignancy (p = 0.02). Overall, odds ratios (ORs) for malignancy were higher for older age (OR 1.06, p = 0.04), male sex (OR 4.34, p = 0.02), RS3PE patients (OR 4.83, p = 0.01), and patients with extremity edema (OR 4.83, p = 0.01). RS3PE patients had higher inflammation levels and associated factors of malignancy than SNRA patients. Patients who are older, male, with extremity edema, or with RS3PE should be screened for malignancies.

Seronegative Arthritis as a Complication of Whipple’s Disease

Whipple’s disease (WD) is an uncommon cause of seronegative arthritis. WD is known for its gastrointestinal symptoms of diarrhea, weight loss, and abdominal pain. However, arthritis may precede gastrointestinal symptoms by 6 to 7 years. We describe a case of an 85-year-old Caucasian male with multiple joint complaints, not responsive to traditional treatments for conditions such as rheumatoid arthritis and osteoarthritis. We suggest that WD be considered for seronegative arthritis especially affecting large joints.

Chronic Myelomonocytic Leukemia Presenting With Polyserositis and Seropositivity for Rheumatoid Arthritis

Chronic myelomonocytic leukemia (CMML) is a rare clonal stem cell disorder associated with clinical and pathologic of myelodysplasia and myeloproliferation. Systemic autoimmune/inflammatory disorders (SAID) and polyserositis have been associated with CMML. These manifestations can be observed concomitantly, shortly before diagnosis or anytime along the course of illness. We report a case of myeloproliferative CMML who presented with polyserositis and positive serology for rheumatoid arthritis. Retrospective studies of myelodysplasia/CMML have reported 15% to 25% incidence of SAID. The most commonly observed disorders include systemic vasculitis, connective tissue diseases, polychondritis, seronegative arthritis, and immune thrombocytopenia. SAID does not confer adverse prognosis in retrospective studies. Polyserositis is less common; this may result from leukemic infiltrate or result from autoimmunity. Treatment of serositis includes steroids and cytoreductive agents. Serositis may confer poor prognosis and hypomethylating therapy may improve the outcome.