September18, Unitedkingdom  2021 

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Abstract Volume: 2 Issue: 3 ISSN:

Fatal complications of rheumatoid arthritis and associated diseases of the lungs – A postmortem clinicopathologic study of 147 patients

Miklós Bély*, Ágnes Apáthy1

 

1.Department of Rheumatology, St. Margaret Clinic Budapest, Hungary.


*Corresponding Author: Dr. Miklós Bély M.D., Ph.D., D.Sc. Acad. Sci. Hung.

Hospital of the Order of the Brothers of Saint John of God, Budapest, Hungary, Department of Pathology.

H- 1027 Budapest, Frankel L. 17-19, Hungary
 

Received Date: February 18, 2021

Publication Date: March 01, 2021

 

Abstract

Background

Systemic autoimmune vasculitis (sAV), systemic AA amyloidosis (sAAa) generalized lethal septic infection (SI) with or without purulent arthritis (PA) are the most important complications of rheumatoid arthritis (RA), which may involve the lungs.

In addition to these complications of RA (sAVsAAaSI), a wide spectrum of lung diseases multifocal pneumonia (mfPn), tuberculosis (TB) or malignant tumors (mTu) may associate with RA.

This study aimed to determine that group of patients in which the risk of complications and associated diseases is the highest based on the age, sex of patients, onset and duration of RA, furthermore to evaluate the proportion of mortality and missed diagnosis.

Patients and Methods

147 random autopsy patients with RA were studied.  RA was confirmed clinically according to the criteria of the ACR.

The prevalence of complications and associated diseases of the lungs was confirmed by a detailed review of extensive histological material.

Results

Elderly (especially female) patients were more likely to be affected by sAV than younger or male patients.  sAV complicated RA in 31 (21.08%) of 147 patients and led directly to death in 19 (12.92% of 147).  sAV was clinically recognized in 5 (16.13 %) of 31 patients.

The ratio of diagnosed (n=5) and missed (n=26) cases of sAV was: 0.19 (5:26 of 31).

Amyloidosis developed in both sexes, and at any time in the course of the disease.  sAAa complicated RA in 34 (23.13%) of 147 patients and was fatal in 17 (11.56 % of 147) patients.  sAAa with uremia was clinically diagnosed in 9 (26.47 %) of 34 patients.

The ratio of diagnosed (n=9) and missed (n=25) cases of sAAa was: 0.36 (9:25 of 34).

Fatal SI with or without PA developed in both sexes, and at any time in the course of the disease.  SI complicated RA in 22 (14.97 %) of 147 patients, in association with PA in 11 (50.0 %) of 22 patients.  SI was clinically diagnosed in 9 (0.41 %) of 22, and PA in 5 (45.45 %) of 11 cases. The ratio of diagnosed (n=9) and missed  (n=13) fatal cases of SI was: 0.69 (9:13 of 22), and it was: 0.83 (5:6 of 11) in cases of PA.

The prevalence of mfPn was higher in our elderly female and male autoimmune patients with impaired immune function than in younger.

Distinct forms of mfPn with fatal outcomes were detected in 28 (19.05 %) of 147 patients.  mfPn was clinically diagnosed in 19 (67.86 %) of 28 patients.

The ratio of diagnosed (n=19) and missed (n=9) fatal cases of mfPn was: 2.11 (19:9 of 28).

Conclusions:

Complications of RA (sAVsAAaSI) and/or allied disorders of the lungs (mfPnTBmTu) were present in 111 (75.51 % of 147) patients, accompanying RA in both sexes and at any time in the course of RA.

Only 36 (24.49 % of 147) patients were found without complications or allied disorders of the lungs, which died of consequences of atherosclerosis (Ath) or accidental (post-operative embolia in one case).

The ratio of diagnosed and missed cases was very bad in case of sAV (0.19) in contrast to sAAa (0.36), SI (0.69) or PA (0.83).

Between associated diseases of the lungs, the TB (0.11) topped the list as the most dangerous and life-threatening diseases compared to the mTu (0.75) or mfPn (2.11).

Keywords

Rheumatoid arthritis, autoimmune vasculitis, AA amyloidosis, lethal septic infection, purulent arthritis, multifocal pneumonia, tuberculosis malignant tumors.

Fatal complications of rheumatoid arthritis and associated diseases of the lungs – A postmortem clinicopathologic study of 147 patients

Abbreviations:

RA – Rheumatoid Arthritis

ACR American College of Rheumatology

sAV –  systemic Autoimmune Vasculitis

pAV –  pulmonary Autoimmune Vasculitis

sAAa –  systemic AA amyloidosis

pAAa –  pulmonary AA amyloidosis (amyloid A deposits in the lungs)

RhV – Rheumatoid Vasculitis

SI –  Septic Infection with fatal outcome

PA –  Purulent Arthritis

IPn –  Interstitial Pneumonitis

mfPn – multifocal Pneumonia with fatal outcome

purBr – purulent Bronchitis or bronchiolitis

BrPn – BronchoPneumonia

InfPn – InfarctPneumonia

OcclPn – OcclusivePneumonia

TB – Tuberculosis

fTB – fibrous TB (inactive tuberculotic scar without miliary dissemination)

fcTB – fibrocaseous TB (fibrocaseous tubercle with or without miliary dissemination)

mTB – miliary TB (active TB with miliary dissemination)

mTumalignant Tumor

CaBrAlvBronchoalveolar Carcinoma

AthAtherosclerosis

HT Hypertension

c – Coefficient of colligation (coefficient of association); range of values from”-1” to “+1”: „-1” indicates a perfect inverse (negative) relationship, „0” indicates no relationship, and „+1” means a perfect positive correlation

SD – Standard Deviation

ND – No Data

NS – Not Sgificant

HEHematoxylin-Eosin staining

 

Introduction

Systemic autoimmune vasculitis (sAV), systemic AA amyloidosis (sAAa) generalized lethal septic infection (SI) are the most important complications of rheumatoid arthritis (RA), which may involve the lungs (1).

In addition to these complications of RA (sAV, sAAa, SI), a wide spectrum of lung diseases may associate with RA (2).

The aim of this study was to determine the prevalence of sAV, sAAa, and lethal SI in RA, to appraise the involvement of bronchial or pulmonary blood vessels by autoimmune vasculitis (pAV), to identify the amyloid A deposition on different tissue structures of the lungs (pAAa), and to characterize the histological features of SI in lungs.

The authors estimated the spectrum of associated diseases (related or not related to RA) in the lungs and identified the group of patients in which the risk of complications and associated diseases is the highest, compared to the patient cohort’s without complications and/or associated diseases, based on the age, sex of patients, onset and duration of RA.

 

Patients and Methods

147 random autopsy patients with RA were studied (3,4)RA was confirmed clinically according to the criteria of the American College of Rheumatology (ACR) (5).

The prevalence of sAV and pAV was confirmed by a detailed review of extensive histological material in agreement with the recommendations of the Consensus Conference (2013) (6).

Amyloid A deposition was diagnosed histologically according to Romhányi (7) by a modified (more sensitive) Congo red staining (8).  Amyloid A deposits were identified in serial sections by immunohistochemical and histochemical methods (9, 10).  The prevalence and severity of amyloid A deposition were evaluated microscopically with an Olympus BX51 polarizing microscope.

Lethal cases of SI and distinct forms of multifocal inflammation (mfPn) of the lungs, such as purulent bronchitis or bronchiolitis (purBr), bronchopneumonia (BrPn), infarctpneumonia (InfPn), occlusive (obliterative or obstructive necrotizing) pneumonia (OcclPn), rheumatoid pneumonia (RhPn), interstitial pneumonia (IPn), tuberculosis (TB) (2), furthermore primary malignant tumors of the lungs (mTu) were determined at autopsy and analyzed retrospectively, confirmed microscopically by a detailed review of extensive histological material, reviewing retrospectively all available clinical and pathological reports.

From each patient, a total of 50-100 tissue blocks of 12 organs (heart, lung, liver, spleen, kidneys, pancreas, gastrointestinal tract, adrenal glands, skeletal muscle, peripheral nerve, skin and brain) were studied microscopically.

Demographics of different patient cohorts were compared with the Student (Welch) t-probe (11).

 

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Volume 2 Issue 3 March 2021

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