scholarly journals Focal sialadenitis in patients with ankylosing spondylitis and spondyloarthropathy: a comparison with patients with rheumatoid arthritis or mixed connective tissue disease

2001 ◽  
Vol 60 (8) ◽  
pp. 744-749 ◽  
Author(s):  
L M J Helenius
Keyword(s):  
Rheumatoid Arthritis ◽  
Ankylosing Spondylitis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease

AB1274 STIFF SPINE AND A WEAK HEART: A CASE OF LONG STANDING ANKYLOSING SPONDYLITIS DEVELOPING PULMONARY ARTERIAL HYPERTENSION SECONDARY TO MIXED CONNECTIVE TISSUE DISEASE, CONFERRING POOR PROGNOSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1928-1929
Author(s):  
C. Dharmapalaiah ◽  
B. Ms ◽  
P. Sn
Keyword(s):  
Ankylosing Spondylitis ◽  
Pericardial Effusion ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Case Reports ◽  
Peripheral Arthritis ◽  
Small Joint ◽  
Pulmonary Arterial ◽  
Salt And Pepper

Background:Spondyloarthritides (SpA) and Connective Tissue Diseases (CTD) are considered distinct entities with diverse clinical features and genetic characteristics. There are very few case reports1of SpA coexisting with CTDs like Lupus, Scleroderma and Morphoea. Drugs used in treating SpA like Sulphasalazine and anti TNF drugs can also induce CTD.Objectives:We report a case of a patient with eleven years history of Ankylosing Spondylitis (AS), presenting with Mixed Connective Tissue Disease (MCTD) and Pulmonary Arterial Hypertension (PAH) constituting a therapeutic challenge.Methods:A 36 year old gentleman was diagnosed with AS at the age of 25 years, fulfilling the ASAS criteria (chronic inflammatory back pain, sacroiliitis on radiograph, HLAB27 positive). He was treated with NSAIDs, Sulphasalazine (SSZ) and physical therapy since 2008. There was gradual progression of his arthritis with high BASDAI along with recurrent anterior uveitis. He was treated with 5 doses of IV Infliximab 3mg/kg, between 2017 and early 2018. In May 2018, following further Infliximab he developed a serum sickness like reaction which was thought to be HACA response to Infliximab. He responded to IV hydrocortisone and antihistamines and Infliximab was discontinued.In February 2019 he developed severe flare up of peripheral arthritis. He was treated with Injection Adalimumab 40mg every 2 weeks along with Latent TB prophylaxis with Isoniazid and Rifampicin. He received 4 doses to no effect and was discontinued.In April 2019 Methotrexate (MTX) was added for peripheral arthritis. He discontinued both MTX and SSZ in July 2019 due to inefficacy. Peripheral arthritis responded well to Leflunomide that was started in September 2019.There was an unexpected turn of events in October 2019, when he was admitted with severe dyspnoea and cough with new onset raynauds, skin tightening over forearms and nape of neck with salt and pepper appearance of skin at these sites (Images). He was hypoxic requiring oxygen support. Echocardiogram showed moderate pericardial effusion and pulmonary hypertension (PASP 60mmHg), dilated right heart and pulmonary artery. Pulmonary embolism was excluded on a CT pulmonary angiogramFigure 1.Image 1, 2 – “salt and pepper” appearance of skin over the wrist and nape of neck, small joint arthritisFigure 2.Image 1, 2 – “salt and pepper” appearance of skin over the wrist and nape of neck, small joint arthritisResults:Investigations revealed 3+ ANA speckled pattern, anti RNP/ Sm 3+, Rheumatoid Factor negative. CRP 45.7u/l, Hemogram, renal and liver function tests were normal.Cardiac MRI showed minimal pericardial effusion with mildly dilated right ventricle, non-dilated left ventricle with LVEF (~44%).Right heart catheterization confirmed PAH with Mean PAP 58mmHg, LVEDP 8mmHg, PCWP 15mmHgA diagnosis of Mixed Connective Tissue Disease (MCTD) was made, associated with PAH and pericardial effusion.He was started on Ambrisentan and Tadalafil for PAH. Hydroxychloroquine and Mycophenolate Mofetil were also added in view of the PAH being associated with CTD. The additional pericardial effusion confers a poor prognosis.Conclusion:Association of Spondyloarthritides and Connective Tissue Disease is rare. There are very few case reports of their chance association, especially MCTD2. Our patient had been exposed to Sulphasalazine, Infliximab, Adalimumab and Isoniazid, all with a potential to induce an auto immune CTD. MCTD features have persisted despite drug withdrawal. This case may suggest routinely checking for ANA in SpA patients prior to initiating anti TNF drugs.References:[1]Brandt J, Maier T, Rudwaleit M et al. Co-occurrence of spondyloarthropathy and connective tissue disease: Development of Sjögren’s syndrome and mixed connective tissue disease (MCTD) in a patient with ankylosing spondylitis. Clinical and experimental rheumatology. 2002;20:80-4.[2]Lee JK, Jung SS, Kim TH, Jun JB, Yoo DH, Kim SY. Coexistence of ankylosing spondylitis and mixed connective tissue disease in a single patient. Clin Exp Rheumatol. 1999;17:263.Disclosure of Interests:None declared

Connective tissue disease

2019 ◽  
pp. 281-326
Author(s):  
Gavin Spickett
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Raynaud’S Phenomenon ◽  
Connective Tissue Diseases ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of connective tissue diseases. It covers a range of rheumatic disorders, from rheumatoid arthritis to Raynaud’s phenomenon, and also covers the undifferentiated diseases, overlap syndromes, and mixed connective tissue disease.

Rheumatology

2014 ◽  
Author(s):  
Murray Longmore ◽  
Ian B. Wilkinson ◽  
Andrew Baldwin ◽  
Elizabeth Wallin
Keyword(s):  
Rheumatoid Arthritis ◽  
Back Pain ◽  
Ankylosing Spondylitis ◽  
Connective Tissue ◽  
Septic Arthritis ◽  
Reactive Arthritis ◽  
Mixed Connective Tissue Disease ◽  
Connective Tissue Diseases ◽  
Locomotor System ◽  
System P

The rheumatological historyAssessing the locomotor systemRheumatological investigationsBack painOsteoarthritis (oa)Septic arthritisRheumatoid arthritis (ra)Crystal arthropathies:Goutcppd arthropathySpondyloarthritides:Ankylosing spondylitis (as)Enteropathic, psoriatic and reactive arthritisAutoimmune connective tissue diseases:Mixed connective tissue disease...

Distal Renal Tubular Acidosis Associated with Mixed Connective Tissue Disease and Hypothyroidism

2018 ◽  
Vol 30 (2) ◽  
pp. 76-78
Author(s):  
Razib Kumar Saha ◽  
Md Azizul Haque ◽  
Mohammad Hasan Tarik
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Renal Tubular Acidosis ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Distal Renal Tubular Acidosis ◽  
Acid Transport ◽  
Systemic Lupus ◽  
Renal Tubular

Renal tubular acidosis (RTA) is caused by defect in renal tubular acid transport. Sjogren’s syndrome, rheumatoid arthritis, systemic lupus erythematosus and autoimmune hepatitis are the most common autoimmune causes of distal RTA. We are reporting a case of distal renal tubular acidosis associated with mixed connective tissue disease and hypothyroidism presenting as recurrent hypokalemic paralysis.TAJ 2017; 30(2): 76-78

scholarly journals The Efficacy and Safety of Rituximab in a Patient with Rheumatoid Spondylitis

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Şenol Kobak ◽  
Ahmet Karaarslan ◽  
Fahrettin Oksel
Keyword(s):  
Rheumatoid Arthritis ◽  
Ankylosing Spondylitis ◽  
Connective Tissue ◽  
Partial Response ◽  
Connective Tissue Disease ◽  
Complete Response ◽  
Response To Treatment ◽  
Tnf Alpha ◽  
Efficacy And Safety ◽  
Proven Efficacy

Rheumatoid arthritis (RA) is considered as a connective tissue disease while ankylosing spondylitis (AS) is a prototype of spondyloarthritis. These diseases are seen concomitantly only very rarely. Also, rituximab has proven efficacy in the treatment of RA while its role in the treatment of AS is unclear. In this presentation, the concomitant presence of RA and AS in a 43-year-old male patient as well as the efficacy and safety of rituximab is discussed. Rituximab was given due to lack of response to treatment with anti-TNF-alpha. Evaluations made at the 6th and 12th months of treatment showed complete response for RA and partial response for AS.

The patient with rheumatoid arthritis, mixed connective tissue disease, Sjögren syndrome, or polymyositis

2015 ◽  
Author(s):  
Lesley-Anne Bissell ◽  
Dwomoa Adu ◽  
Paul Emery
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Renal Disease ◽  
Immune Complex ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Sjögren Syndrome ◽  
Proliferative Glomerulonephritis ◽  
Sjogren Syndrome ◽  
Mesangial Proliferative Glomerulonephritis

Renal disease is a well-recognized cause of ill health and death in rheumatoid arthritis. Three broad categories of renal disease occur. The first—and by far the most common—arises from the nephrotoxicity of the drugs used in the treatment of arthritis, particularly with non-steroidal anti-inflammatory drugs. Disease-modifying antirheumatic drugs such as gold and D-penicillamine may lead to proteinuria and a glomerulonephritis in 10–30% of patients. Ciclosporin is associated with significant nephrotoxicity and hypertension. A second major but diminishing cause of renal disease in rheumatoid arthritis is amyloidosis. Thirdly, rheumatoid arthritis may be associated with the development of glomerulonephritis. The main types described are a mesangial proliferative glomerulonephritis with or without immunoglobulin A deposits, a membranous nephropathy, and a focal segmental necrotizing glomerulonephritis of the vasculitic type.Renal disease in mixed connective tissue disease and polymyositis is infrequent, but the former can be associated with a membranous and mesangial proliferative glomerulonephritis.Sjögren syndrome is rarely associated with clinically significant renal disease, but patients can present with proteinuria, acidosis, or hyperchloraemia. Interstitial nephritis and immune complex glomerulonephritis reflect the exocrinopathy and circulating immune complex disease pathognomonic of Sjögren syndrome. Evidence for effective treatment of the renal complications is lacking. Corticosteroids and cyclophosphamide are most commonly used, with newer biological drugs, such as rituximab, showing promise.

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