scholarly journals Mycobacterium intracellulare Infection Mimicking Progression of Scleroderma

2017 ◽  
Vol 2017 ◽  
pp. 1-3
Author(s):  
Simon Krabbe ◽  
Merete Engelhart ◽  
Sören Thybo ◽  
Søren Jacobsen
Keyword(s):  
Case Report ◽  
Listeria Monocytogenes ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Connective Tissue Disorder ◽  
High Dose ◽  
Esophageal Dysmotility ◽  
Mycobacterium Intracellulare ◽  
The Right ◽  
Inflammatory Changes

This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud’s phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare. While she was receiving high-dose prednisolone, as worsening of her connective tissue disease was suspected to be the cause of the inflammatory changes, she had Listeria monocytogenes meningitis and was hospitalized for several weeks, but she recovered from this without sequelae. After Mycobacterium intracellulare infection was diagnosed, she was treated with clarithromycin and rifampicin. Her skin manifestations, arthralgias, and fatigue improved considerably, and the wounds of the right leg healed, unfortunately with significant scarring. Immunodeficiency testing was unremarkable. In summary, an infection with Mycobacterium intracellulare was mistaken for an unusually severe progression of scleroderma.

scholarly journals Sequential bilateral Behçet’s neuroretinitis associated with prepapillary vitreous exudate: case report

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Imen Ksiaa ◽  
Safa Ben Aoun ◽  
Sourour Zina ◽  
Dhouha Nefzi ◽  
Sana Khochtali ◽  
...  
Keyword(s):  
Case Report ◽  
Single Case ◽  
Signal Loss ◽  
Fiber Layer ◽  
Swept Source ◽  
Single Case Report ◽  
Shadowing Effect ◽  
Eye Involvement ◽  
The Right ◽  
Inflammatory Changes

Abstract Objective To describe a case of Behçet disease (BD) uveitis manifesting with sequential bilateral neuroretinitis associated with prepapillary inflammatory vitreous exudate (PIVE). Material and methods A single case report documented with multimodal imaging. Results A 37-year-old man developed neuroretinitis with associated PIVE in the left eye. He was diagnosed with ocular toxoplasmosis and treated accordingly based on positive serologic testing and negative work-up for other entities, including BD. The disease course was favorable, but 1 year later a similar neuroretinitis developed in the right eye. Extraocular features of BD became evident only at the time of the second eye involvement, and the patient received corticosteroid and immunosuppressive therapy. Swept source (SS) OCT showed at the acute phase in both eyes a typical “mushroom-shaped” prepapillary hyperreflectivity of the PIVE. SS OCT angiography (OCTA) demonstrated a corresponding prepapillary hypointense area due to shadowing effect, decreasing in size while scanning deeper layers. It also detected peripapillary retinal hypervascularity in both eyes and a sectoral area of flow signal loss in the first involved left eye. Visual acuity improved following the resolution of the PIVE and associated acute inflammatory changes in both eyes. The left eye showed residual optic disc pallor and retinal nerve fiber layer defects. Conclusion Sequential bilateral neuroretinitis associated with PIVE may occur before other clinical features of BD become evident. SS OCT and OCTA can provide useful information for the diagnosis and management of this rare, but typical, ocular manifestation of BD uveitis.

Refractory eosinophilic fasciitis successfully treated with infliximab: A case report

2021 ◽  
pp. 239719832110043
Author(s):  
Paulina Śmigielska ◽  
Justyna Czarny ◽  
Jacek Kowalski ◽  
Aleksandra Wilkowska ◽  
Roman J. Nowicki
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Treatment Failure ◽  
Immunosuppressive Drugs ◽  
Unknown Etiology ◽  
High Dose ◽  
Eosinophilic Fasciitis ◽  
Line Treatment ◽  
First Line ◽  
First Line Treatment

Eosinophilic fasciitis is a rare connective tissue disease of unknown etiology. Therapeutic options include high-dose corticosteroids and other immunosuppressive drugs. We present a typical eosinophilic fasciitis case, which did not respond to first-line treatment, but improved remarkably after infliximab administration. This report demonstrates that in case of initial treatment failure, infliximab might be a relatively safe and effective way of eosinophilic fasciitis management.

scholarly journals Anti-MDA5 Antibody Dermatomyositis Overlap with Systemic Lupus Erythematosus: A Case Report and Review of the Literature

2016 ◽  
Vol 10 (1) ◽  
pp. 122-128 ◽  
Author(s):  
Emily C. Milam ◽  
Jacobo Futran ◽  
Andrew G. Franks Jr.
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Clinical Phenotype ◽  
Review Of The Literature ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Clinical Presentations

Background: Dermatomyositis (DM) is an autoimmune connective tissue disease that primarily targets the muscle, skin, and lungs. Many patients have autoantibodies that correspond to distinct clinical phenotypes. Melanoma differentiation-associated gene 5 (anti-MDA5) antibody, a specific antibody that targets the melanoma differentiation-associated gene 5 (MDA5), has been reported in DM cases and is significant for a distinct cutaneous presentation and rapidly progressive interstitial lung disease. Objective: Herein, we describe a patient with DM with a positive anti-MDA5 antibody and characteristic clinical phenotype, who subsequently developed coexisting systemic lupus erythematosus (SLE). A diagnosis of SLE was supported by his clinical phenotype, positive serologies, hypocomplementemia, and progression to glomerulonephritis and lupus cerebritis, features of which fulfilled the American College of Rheumatology criteria for SLE. Conclusion: DM is known to overlap with other autoimmune diseases, including SLE, and coexistence can lead to a wide variety of clinical presentations. SLE overlapping with anti-MDA5 positive DM may present with distinct clinical features.

scholarly journals A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

scholarly journals Distal Renal Tubular Acidosis as complication of Mixed Connective Tissue Disorder with autoimmune hypothyroidism- a case report

2016 ◽  
Vol 27 (1) ◽  
pp. 37-39
Author(s):  
Md Daharul Islam ◽  
SM Tajdit Rahman ◽  
Khaleda Akter ◽  
Md Azizul Hoque
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Renal Tubular Acidosis ◽  
Connective Tissue Disorder ◽  
Distal Renal Tubular Acidosis ◽  
Undifferentiated Connective Tissue Disease ◽  
Acid Transport ◽  
Autoimmune Hypothyroidism ◽  
Renal Tubular ◽  
Mixed Connective Tissue Disorder

Renal tubular acidosis (RTA) is a constellation of syndromes arising from different derangements of tubular acid transport. Multiple associations have been established with renal tubular acidosis. We report a case of distal renal tubular acidosis which may be associated with undifferentiated connective tissue disease in which patient is also having autoimmune hypothyroidism along with that is not very common.Bangladesh J Medicine Jan 2016; 27(1) : 37-39

A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

The Pulmonary Histopathology of Anti-KS Transfer RNA Synthetase Syndrome

2015 ◽  
Vol 139 (1) ◽  
pp. 122-125 ◽  
Author(s):  
Frank Schneider ◽  
Rohit Aggarwal ◽  
David Bi ◽  
Kevin Gibson ◽  
Chester Oddis ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Disorder ◽  
Transfer Rna ◽  
Organizing Pneumonia ◽  
Antisynthetase Syndrome ◽  
Pulmonary Manifestations

Context The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti–asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. Objective To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. Design We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. Results The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. Conclusions Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS–antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

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