PL-7 Antisynthetase Syndrome in Association with Sjögren’s, Systemic Lupus Erythematosus, and Rheumatoid Arthritis

Jawaid, Mehrin; Ross, Yael; Kamran, Muhammad

doi:10.1155/2020/4736476

Cited by 3 publications

(2 citation statements)

References 16 publications

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“…According to Kurata et al, aortic aneurysm is a rare cardiac complication of lupus [ 24 , 29 ]. Diagnosis and treatment of cardiopulmonary involvement in CTD may be delayed due to its ambiguity of presenting symptoms and is associated with increased morbidity and mortality and poor survival [ 30 ]. Therefore, careful attention should be paid to identify patients with CTD to identify features of cardiopulmonary involvement.…”

Section: Discussionmentioning

confidence: 99%

Coexistence of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Sjogren Syndrome, Antiphospholipid Syndrome, and Ankylosing Spondylitis

Khasru

Siddiq²,

Sayeeduzzaman

et al. 2021

Case Reports in Rheumatology

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A 37-year-old Bangladeshi woman presented with low back and several joints pain and swelling for months together; there was significant morning stiffness for more than two hours. Repeated abortions, dry eye, hair fall, photosensitivity, and oral ulcer were the additional complaints. Clinical examination unveiled asymmetrical peripheral and both sacroiliac joint tenderness, positive modified Schober’s test, and limited chest expansion. Schirmer’s test was positive. The history of rheumatoid arthritis (RA) and ankylosing spondylitis (AS) among 1st-degree relatives was also significant. Biochemical analysis revealed pancytopenia, raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), and mild microscopic proteinuria. The patient was seropositive for rheumatoid factor (RF), antibodies against cyclic citrullinated peptides (anti-CCP), antinuclear antibody (ANA), anti-Sm antibody, anti-Sjögren’s-syndrome-related antigen A and B (anti-SSA/SSB), antiphospholipid (aPL-IgG/IgM), and HLA B27; however, serum complement (C3 and C4) levels were normal. Basal cortisol level measured elevated. Besides, X-ray and MRI of lumbosacral spines demonstrated sacroiliitis. There was radiological cardiomegaly, echocardiography unveiled atrial regurgitation, and ascending aorta aneurysm. Based on the abovementioned information, RA, AS, and systemic lupus erythematosus (SLE) have been diagnosed. Moreover, the patient developed Sjogren’s syndrome (SS), antiphospholipid lipid syndrome (APS), Cushing syndrome, ascending aorta aneurysm, and atrial regurgitation. Her disease activity score for RA (DAS28), DAS for AS (ASDAS), SLE disease activity index (SLEDAI), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) scores were 3.46, 2.36, 23, and 5, respectively. The patient received hydroxychloroquine (200 mg daily), pulsed cyclophosphamide, prednisolone (20 mg in the morning), and naproxen 500 mg (twice daily). To our best knowledge, this is the first report documenting RA, AS, and SLE with secondary SS and APS.

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Section: Discussionmentioning

confidence: 99%

Coexistence of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Sjogren Syndrome, Antiphospholipid Syndrome, and Ankylosing Spondylitis

Khasru

Siddiq²,

Sayeeduzzaman

et al. 2021

Case Reports in Rheumatology

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“…Hervier and Lambert et al reported an interesting rare case of overlap syndrome with PL-7 ASS, Sjogren's, systemic lupus erythematosus and RA with evolving symptoms over time. 8,[13][14][15] Anti-synthetase syndrome has often been reported in overlap with polymyositis, dermatomyositis, systemic sclerosis, Sjogren's and RA.…”

Section: Discussionmentioning

confidence: 99%

The anti-synthetase syndrome in a type 1 diabetes

Sajjan¹,

Pahuja²,

Ponvijaya³

et al. 2020

Int J Res Med Sci

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Anti-synthetase syndrome is an autoimmune disease associated with interstitial lung disease (ILD), dermatomyositis and polymyositis. It has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with dermatomyositis. 37-year-old male, known case of type 1 diabetes mellitus, came with complaints of: generalized anasarca, pain in both knees, ankles, wrist and small joints of fingers. He also had dyspnoea on exertion, chronic non-productive cough, and fever off and on, all for 1 month. Initially all involvement was attributed to diabetes. For joint pain an antinuclear antibodies (ANA) was sent. He turned out to be anti-Jo1, antibody positive. Rash on hands was diagnosed by dermatologist as, mechanic’s hand, hence diagnosed as an inflammatory myopathy with dermatomyositis anti-synthetase syndrome. Patient was successfully treated with immunosuppressants and supportive treatment and responded to tablet Prednisolone and Mycophenolate mofetil. The patient had one major and 2 minor criteria-ILD, arthritis and Mechanic’s hand and anti-Jo 1 antibody positive. Thus, diagnosed as anti-synthetase syndrome with type 1 diabetes mellitus.

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Rare immunofluorescence patterns of autoantibodies on HEp-2 cells defined by ICAP identify different autoimmune diseases in the absence of associated specificities: a Spanish multicentre study

et al. 2021

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Objectives ANA are the most extensively used test for the diagnosis of systemic autoimmune diseases. However, testing by indirect immunofluorescence assays (IIFAs) on HEp-2 cells, the gold standard test, is time-consuming and needs expertise. Thus there is a trend to replace it with other automated solid-phase assays directed against specific ANA. Nonetheless, the Hep-2 cell is an autoantigen array and ANA have been classified into 29 types, some of them with no clear association with a specificity to be detected. It is especially in these uncommon patterns where no clinical relationship is found and no antigenic specificity is detected. Here we retrospectively collected clinical data from patients with confirmed uncommon HEp-2 IIFA patterns to search for an associated clinical condition. Methods We conducted an observational retrospective study including 608 patients with organ-specific and non-organ-specific autoimmune diseases (OSADs and NOSADs, respectively) with a confirmed rare pattern of ANA detected by IIFA on HEp-2 cells in the routine practice of the Spanish European Autoantibodies Standardization Initiative laboratories. Inclusion criteria are the existence of a minimum follow-up of 2 years and the availability of clinical data. Results Nuclear patterns were more frequent in SLE (P = 0.001) and SS (P = 0.001), whereas the cytoplasmic ones were significantly higher in SSc (P = 0.022) and inflammatory myositis (P = 0.016). Mitotic patterns did not show any preferences for a specific disease and 62.7% of them corresponded to the nuclear mitotic apparatus pattern (AC-26). The most frequent NOSADs in patients with the AC-26 pattern were SLE (28.6%), SS (11.9%) and RA (11.9%). The cytoplasmic HEp-2 IIFA patterns were equally distributed in both groups of patients. In the OSAD patients there was no predominant pattern, except for AC-6 in primary biliary cholangitis due to Sp-100 antibodies (P < 0.001). Conclusion Detection of infrequent ANA might be a unique finding with no disease-associated specificities and could lead to the suspicion of an autoimmune disease.

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PL-7 Antisynthetase Syndrome in Association with Sjögren’s, Systemic Lupus Erythematosus, and Rheumatoid Arthritis

Cited by 3 publications

References 16 publications

Coexistence of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Sjogren Syndrome, Antiphospholipid Syndrome, and Ankylosing Spondylitis

Coexistence of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Sjogren Syndrome, Antiphospholipid Syndrome, and Ankylosing Spondylitis

The anti-synthetase syndrome in a type 1 diabetes

Rare immunofluorescence patterns of autoantibodies on HEp-2 cells defined by ICAP identify different autoimmune diseases in the absence of associated specificities: a Spanish multicentre study

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