Relapsing polychondritis: state-of-the-art review with three case presentations

ABSTRACT

Background

Relapsing polychondritis (RPC) is a complex immune-mediated systemic disease affecting cartilaginous tissue and proteoglycan-rich organs. The most common and earliest clinical features are intermittent inflammation involving the auricular and nasal regions, although all cartilage types can be potentially affected. The life-threatening effects of rpc involve the tracheobronchial tree and cardiac connective components. Rpc is difficult to identify among other autoimmune comorbidities; diagnosis is usually delayed and based on nonspecific clinical symptoms with limited laboratory aid and investigations. Medications can vary, from steroids, immunosuppressants, and biologics, including anti-tnf alpha antagonist drugs.

Method

Information on updated etiology, clinical symptoms, diagnosis, and treatment of rpc has been obtained via extensive research of electronic literature published between 1976 and 2019 using PubMed and medline databases. English was the language of use. Search inputs included ‘relapsing polychondritis,’ ‘polychondritis,’ ‘relapsing polychondritis symptoms,’ and ‘treatment of relapsing polychondritis.’ Published articles in English that outlined and reported rpc’s clinical manifestations and treatment ultimately met the inclusion criteria. Articles that failed to report the above and reported on other cartilaginous diseases met the exclusion criteria.

Result

Utilizing an extensive overview of work undertaken in critical areas of RPC research, this review intends to further explore and educate the approach to this disease in all dimensions from pathophysiology, diagnosis, and management.

Conclusion

RPC is a rare multi-systemic autoimmune disease and possibly fatal. The management remains empiric and is identified based on the severity of the disease per case. The optimal way to advance is to continue sharing data on RPC from reference centers; furthermore, clinical trials in randomized control groups must provide evidence-based treatment and management. Acquiring such information will refine the current knowledge of RPC, which will improve not only treatment but also diagnostic methods, including imaging and biological markers.

KEYWORDS:

• Relapsing polychondritis
• auricular chondritis
• etiology
• diagnosis
• treatment

MESH:

• polychondritis relapsing/etiology
• polychondritis relapsing/diagnosis
• polychondritis relapsing/drug therapy

Abbreviations

ANA – anti-nuclear antibodies

ANCA - anti-neutrophil cytoplasmic antibodies

CABG - coronary artery bypass grafting

CNS – central nervous system

COPD - chronic obstructive pulmonary disease

CPG - clinical practice guidelines

CRP - C-reactive protein

CT – computer tomography

DES - drug-eluting stents

DMARDs - disease-modifying antirheumatic drugs

EBUS - endobronchial ultrasonography

ECG - electrocardiogram

ESR - erythrocyte sedimentation rate

FDG - F-fluorodeoxyglucose

GCS - glucocorticosteroids

GPA - granulomatosis with polyangiitis

HRCT - high-resolution CT

ITP. - immune thrombocytopenia

MAGIC syndrome - mouth and genital ulcers with inflamed cartilage

MRI - Magnetic resonance imaging

NKT - natural killer T

NSAIDs - non-steroidal anti-inflammatory drugs

NSTEMI - non-ST-elevation myocardial infarction ()

PTCA - percutaneous transluminal coronary angioplasty ()

RPC - relapsing polychondritis

RPDAI - relapsing Polychondritis Disease Activity Index

SLE - systemic lupus erythematosus

TBNA - PET-CT with transbronchial needle aspiration

TNF – tumor necrosis factor

UCTD - undifferentiated connective tissue disease