Pulmonary manifestations of collagen vascular disorders

: Interstitial lung disease (ILD) is the most common form of pulmonary impairment in patients with rheumatoid arthritis (RA). However, patients with RA or other arthritic diseases such as psoriatic arthritis (PsA) or peripheral spondyloarthritis (pSpA) may develop several other pulmonary diseases such as chronic obstructive lung disease (COPD) with a higher risk than patients without arthritis. The article at hand aims at summarizing the current knowledge on the prevalence of pulmonary diseases in the above-mentioned forms of arthritis, the challenges for prevalence studies and detecting pulmonary diseases in patients with arthritis as well as possible treatment options. Dyspnea, cough or other pulmonary symptoms or findings in arthritis patients should prompt gradual diagnostic procedures considering pulmonary manifestations as a major cluster of differential diagnosis. Considering its poor prognosis and morbidity burden, RA-ILD needs to be ruled out. Treatment of manifestations often lacks solid evidencebased guidelines and referrals to specialized centers are often necessary.

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Diagnosis and Treatment of Vascular Disorders: Anglology

The Medical Journal of Australia ◽

10.5694/j.1326-5377.1956.tb57091.x ◽

1956 ◽

Vol 2 (17) ◽

pp. 647-648

Keyword(s):

Diagnosis And Treatment ◽

Vascular Disorders

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Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis

Clinics and Practice ◽

10.3390/clinpract11020035 ◽

2021 ◽

Vol 11 (2) ◽

pp. 235-240

Author(s):

Houari Aissaoui ◽

Kinan Drak Alsibai ◽

Naji Khayath

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Distress Syndrome ◽

Early Stage ◽

Immunosuppressive Treatment ◽

Amyopathic Dermatomyositis ◽

Pulmonary Manifestations ◽

Life Threatening ◽

Adequate Management ◽

Chest Ct Scan

Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management.

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FRI0072 DISCONTINUATION OF DMARD USE IN RHEUMATOID ARTHRITIS PATIENTS WITH LUNG DISEASE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5452 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 612.1-613

Author(s):

S. Pedro ◽

T. Mikuls ◽

J. Zhuo ◽

K. Michaud

Keyword(s):

Rheumatoid Arthritis ◽

Lung Disease ◽

Pulmonary Disease ◽

Treatment Patterns ◽

Dmard Treatment ◽

Increased Risk ◽

Pulmonary Manifestations ◽

Clinical Models ◽

Comorbidity Index ◽

Time Varying Covariates

Background:Pulmonary manifestations such as interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD) are frequent extra-articular features that carry a poor prognosis in Rheumatoid Arthritis (RA). Little data is available on how RA patients (pts) with pulmonary disease are managed in real-world settings.Objectives:To assess treatment patterns and DMARD discontinuation in RA patients with comorbid lung disease in comparison with other RA patients.Methods:The study included RA Patients enrolled in the Forward Databank with ≥1 year observation after 2000 initiating a DMARD. Forward is a large longitudinal rheumatic disease registry in the US. RA patients’ diagnoses were rheumatologist-confirmed, and every 6 months participants completed comprehensive questionnaires regarding symptoms, disease outcomes, medications, and clinical events. Lung disease (LD+) was defined as at least one of the following: emphysema, asthma, bronchitis, COPD, pleural effusion, fibrosis of the lung, “RA lung”, or ILD, the later classified by ICD9 codes (England 2019). DMARDs were categorized hierarchically into four groups: csDMARDs, TNFi and NTNFi (bDMARDs), and tsDMARDs. Percentage of patients who initiated different DMARDs were reported for pts with LD+/LD-. Discontinuation was analyzed by Kaplan Meier (KM) curves, log-ranks tests, and Cox regression models using time-varying covariates. Best models were created using backward selection models (10% probability of removal) and pre-defined clinical models.Results:Of the 21,525 eligible RA patients, 13.8% had LD+ at the time they initiated a DMARD (follow-up: 69,597 pt-yrs (median 1.9 yrs/pt)). LD+ patients tended to have more severe RA outcomes and comorbidities. MTX-monotherapy (48% vs 44%, p<0.001) and NTNFi were initiated more frequently in LD+ pts with lower use of TNFi (Figure). DMARD discontinuation rates were higher among LD+ patients for all DMARD groups, but KM curves were only significantly different for csDMARDs and TNFi. Different HRs for LD+ were found depending on the model used ranging from 1.18 to 1.28, and all models revealed an increased risk of discontinuation for LD+ patients. Compared to csDMARDs, TNFi were more often discontinued (Table). Other variables associated with an increased risk of discontinuation included: HAQ, Rheumatoid Disease (RD) comorbidity index, pain, prior bDMARDs, and csDMARDs.Conclusion:Different DMARD treatment patterns were found for LD+ patients, who tended to initiate more csDMARD and NTNFi and less likely to initiate a TNFi. LD+ patients were at a higher risk of discontinuation irrespectively of the DMARD treatment, but with greater risk for TNF users.References:[1]England BR, et al. Arth Care Res. doi:10.1002/acr.24043.Figure.DMARD treatment initiators by disease groupTable .Cox models for DMARD discontinuation by stepwise (removal probability 10%) and clinical models including DMARD treatment.Model of DMARD persistence*Model 1- Stepwise-Without drugsModel 2 – StepwiseModel 3 - ClinicalLD+ vs LD–1.181.281.20(1.08 - 1.29)(1.13 - 1.45)(1.08 - 1.34)TNF vs csDmard1.321.22(1.08 - 1.63)(1.04 - 1.44)NTNF vs csDmard1.131.13(0.83 - 1.52)(0.90 - 1.41)tsDmard vs csDmard1.301.02(0.65 - 2.60)(0.64 - 1.62)*Best models searched/Clinical adjusted for LD+/LD-, DMARDs, age, sex, education, HAQ disability, RD comorbidity index, smoking, pain, glucocorticoids, year of entry, prior bDMARDs and csDMARDs counts and MRC breath scale.Disclosure of Interests:Sofia Pedro: None declared, Ted Mikuls Grant/research support from: Horizon Therapeutics, BMS, Consultant of: Pfizer, Joe Zhuo Shareholder of: Bristol-Myers Squibb, Employee of: Bristol-Myers Squibb, Kaleb Michaud: None declared

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COVID-19 as the cause of thrombosis: recognising COVID-19 infection in apparently asymptomatic patients

BMJ Case Reports ◽

10.1136/bcr-2020-241027 ◽

2021 ◽

Vol 14 (1) ◽

pp. e241027

Author(s):

Kyle B Varner ◽

Emily J Cox

Keyword(s):

Emergency Department ◽

Direct Result ◽

Abdominal Ct ◽

Call To Action ◽

Asymptomatic Patients ◽

Pulmonary Manifestations ◽

Abdominal Ct Scan ◽

Thrombotic Complications ◽

Work Up ◽

Renal Thrombosis

COVID-19 has serious thrombotic complications in critically ill patients; however, thrombus is not a typical presenting symptom. This case report describes a patient with no respiratory symptoms who presented to the emergency department with abdominal pain. The pain was attributed to renal thrombosis, but the patient was found to have no risk factors for thrombotic disease and subsequent hypercoagulable work-up was unremarkable. Pulmonary manifestations of COVID-19 infection were detected incidentally on the abdominal CT scan and confirmed via PCR test. The patient was isolated and went on to develop mild respiratory failure secondary to COVID-19 infection. This case suggests that unexplained thrombus in otherwise asymptomatic patients can be a direct result of COVID-19 infection, and serves as a call to action for emergency department clinicians to treat unexplained thrombotic events as evidence of COVID-19.

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EP22 A manifestation of severe sarcoid arthropathy in the distal interphalangeal joint

Rheumatology Advances in Practice ◽

10.1093/rap/rkaa052.021 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Rajiv Ark

Keyword(s):

Case Report ◽

Lymph Nodes ◽

Cardiac Sarcoidosis ◽

Treatment Options ◽

Nasal Discharge ◽

Pulmonary Manifestations ◽

Distal Interphalangeal ◽

The Right ◽

Little Finger

Abstract Case report - Introduction In 2011 a gentleman in his 50s presented with nasal blockage and bloody discharge. He was diagnosed with sarcoidosis and after 9 years of failed strategies to control his disease, he developed dactylitis. X-ray of the hands showed severe arthropathy in the distal interphalangeal joints. This case demonstrates an uncommon extrapulmonary manifestation of sarcoidosis. Although most of his follow up was with a respiratory clinic, his main symptoms were not due to interstitial lung disease, highlighting the importance of a multidisciplinary approach. To reduce the need for steroids, several DMARDs were tried illustrating that there are limited treatment options. Case report - Case description This gentleman presented in June 2011 with left epiphora, bloody nasal discharge and fatigue. He had no family history of sarcoidosis and was of Caucasian ethnicity. He was referred by his GP to Ophthalmology and ENT. Septoplasty showed a 95% blockage at the lacrimal sac. A biopsy was performed, and histology showed a nasal sarcoid granuloma. He was referred to the respiratory team who requested a high-resolution CT scan showing sizeable lymph nodes. One inguinal node was biopsied confirming sarcoid granulomas before starting treatment. Calcium was briefly raised, and serum ACE was initially 123. He was started on 40mg of prednisolone for 6 weeks, which was tapered to 20/25mg on alternating days. There was a recurrence of his nasal discharge; steroids were increased again but he developed symptoms of muscle weakness from long term steroid use. He was referred to an interstitial lung disease clinic at a tertiary centre where he was investigated for cardiac sarcoidosis with MRI due to ventricular ectopics. Hydroxychloroquine was started to reduce the steroid use however he developed symptoms of tinnitus, so it was stopped. Methotrexate, Azathioprine and Leflunomide were all trialled to however they did not have any impact on controlling his disease. His Prednisolone was slowly reduced by 1mg a month. When he had recurrence of his symptoms, he was given IV methylprednisolone. Nine years after his first presentation he presented with stiffness of the right thumb base. This progressed to dactylitis and slight fixed flexion deformity of right index finger and left little finger. An x-ray of his hands showed disease in the distal interphalangeal joints bilaterally with severe changes in the left little finger. The effects of long-term steroids led him to request a letter to support early retirement. Case report - Discussion The main rationale for changing treatment options was to reduce the prednisolone dose. Steroids were the only treatment option that showed evidence of controlling his disease when the dose was between 25mg and 40mg a day. Each of the DMARDs that were trialled had a different side effect profile and did not show any evidence of suppressing disease as symptoms recurred. Dose changes later in treatment fluctuated, reflecting a balancing act between disease recurrence and side effects of long-term steroids. There are many extra pulmonary manifestations of sarcoidosis that were investigated in this case. The first being the nasal granuloma, which can occur in sarcoid patients with symptoms of epistaxis, crusting, congestion, and pain. There were granulomatous changes seen in the hila as well as other lymph nodes such as the inguinal region; inguinal lymphadenopathy can lead to pain in the groin area. In addition to this it was important to exclude uveitis with ophthalmology review as he had symptoms of epiphora. Uveitis can be diagnosed in ophthalmological assessment of sarcoid patients in the absence of ocular complaints. Cardiac sarcoidosis was excluded with an MRI at a specialist heart and lung centre due to ventricular ectopics. Cardiac sarcoidosis can lead to heart block, arrhythmias, and congestive cardiac failure. Finally, he developed sarcoid arthropathy, review of his radiological images over time showed extensive damage to the joints of the hand. This gentleman had poor outcomes due to limited treatment options for his disease. Being restricted to long term steroid as the mainstay of treatment led to early retirement due to fatigue and muscle weakness. Conversely, under dosing steroids led to recurrence in symptoms. His disease is still not controlled as shown by an evolving sarcoid arthropathy. Case report - Key learning points An illustration of sarcoid arthropathy is also shown in this case. Sarcoid arthropathy is an uncommon manifestation of the disease primarily affecting joints in the hands and feet. In this case the distal interphalangeal joints and proximal interphalangeal joints were affected. The first symptom of arthropathy was stiffness of the base of the right thumb in 2017, this could fit with an osteoarthritic picture and could be mistaken for it in undiagnosed sarcoidosis. The most severe disease was in the DIP of the left little finger, which is not commonly affected. An oligoarthritic pattern with involvement of the ankle is seen more often. This is also an unusual case of sarcoidosis as there was no family history of the disease and his ethnicity did not predispose him to the condition. He also had a few uncommon extra pulmonary manifestations of sarcoidosis. The importance of a multidisciplinary approach in managing sarcoidosis was demonstrated in this case. Most of his follow up was with a respiratory clinic. However, respiratory symptoms were not the main issue during the patient journey; early ENT and rheumatology input was significant in managing his disease. Although pulmonary lymph nodes were enlarged, they did not affect his lung function.

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Pulmonary echinococcosis: A rare pseudotumour of the lung

Rare Tumors ◽

10.1177/20363613211009769 ◽

2021 ◽

Vol 13 ◽

pp. 203636132110097

Author(s):

Kristina Greimelmaier ◽

Thomas Hager ◽

Vasily Moskalenko ◽

Stefan Mueller-Huelsbeck ◽

Henning Feist ◽

...

Keyword(s):

Central Europe ◽

Hydatid Disease ◽

Cystic Echinococcosis ◽

Frozen Section ◽

Lung Abscess ◽

Thoracic Pain ◽

Primary Lung Carcinoma ◽

Pulmonary Manifestations ◽

Pulmonary Hydatid Disease ◽

Pulmonary Echinococcosis

Cystic echinococcosis is a widely endemic helminthic disease worldwide but occurs only rarely in Central Europe. Humans are infected as ‘aberrant’ hosts by Echinococcus granulosus and develop cysts in numerous different organs. 20%–30% of the affected individuals develop hydatid disease in the lungs with associated complications including pleuritis, lung abscess and pneumothorax. Radiologically, the pulmonary lesions of cystic echinococcosis occasionally pose difficulties in the differential diagnosis of primary lung carcinoma or metastatic disease and vice versa. Herein we report on a case of pulmonary hydatid disease in a 25-year-old Iraqi male presenting with a cystic lesion of the lung associated with thoracic pain and involuntary weight loss. Despite of its rare occurrence in Central Europe, clinicians, radiologists and pathologists should be aware of this entity and its pulmonary manifestations. During frozen section examination, imprint cytology specimens may facilitate the detection of the pathogens.

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