Relapsing Polychondritis
Description
● Overview:
➣ Relapsing polychondritis (RP) is an autoimmune disease that affects cartilaginous and proteoglycan-rich tissues. It is uncommon, severe, episodic, and progressive.
Ears, nose, larynx, trachea, bronchi, blood vessels, heart, eyes, kidneys, and joints are frequently affected.
➣ Background: In 1923, it was initially referred to as "polychondropatia."
• Pearson (1960) provided the current definition.
➣ Epidemiology:
• Annual incidence: approximately 3.5 per million.
• It can strike at any age, but it peaks between the ages of 40 and 50.
• The ratio of women to men is 3:1.
➣ Genetic/Immunological associations:
• RP patients have ↑ HLA-DR4.
• Both humoral and cell-mediated immunity were implicated.
• Type II collagen and matrilin-1 trigger an autoimmune reaction.
• Less than 50% of patients had anti-type II collagen antibodies (non-specific).
➣ Associations: May coexist with systemic illnesses that are not rheumatic.
● Diagnosis
➣based on: bilateral auriculitis, laryngotracheobronchomalacia, asymmetric non-erosive arthritis, and saddle nose deformity (involving the nasal cartilage).
➣ The following criteria were applied:
• McAdam (1976): ≥3 of 6 symptoms.
•Damiani and Levine (1979): Added biopsy and steroid response to their modified criteria.
➣ This patient had a supportive biopsy and met McAdam's ≥3 characteristics.
● Discussion:
➣ The illness is multisystem, progressive, autoimmune, and potentially fatal.
➣Clinical characteristics include an abrupt start and an episodic course.
• Auricular cartilage (earlobe spared, may cause cauliflower ear) is a common site.
• Nasal cartilage: Less frequent, but painful, with scabbing, discharge, blockage, and epistaxis; chronic inflammation results in saddle nose deformity.
• Joints: oligo/polyarthritis that is often asymmetric, seronegative, and non-erosive.
• Involvement of the airways: May resemble TB, asthma, or chronic bronchitis.
➣ Diagnosis: Mostly clinical (characteristics).
• If atypical, a biopsy can be helpful.
➣ Side effects include hearing loss, laryngeal/tracheal stenosis, and airway blockage.
• Pneumonia and other secondary infections; severe arthritis.
➣ Common diagnostic standards:
• Damiani's and McAdam's (most popular).
• For uncommon instances, there are certain further conditions.
● Presentation of the Case:
➣Preliminary Presentation
50-year-old woman with a history of depression, bronchial asthma, diabetes, and systemic hypertension.
➣ Complaints: coughing with little mucoid sputum (6 months), wheezing, and grade 3 dyspnoea (mMRC).
➣Examination: saddle nose deformity, urticaria, and tachypnea.
Bilateral rhonchi plus creeps are audible.
➣Research findings (Table 1):
• ANA: 1+ nucleolar pattern;
• PFT: Obstructive pattern;
• ECG & Echo: Normal;
• ESR: 23 mm/h;
• CRP: 17.7 mg/dL
Respiratory acidosis (ABG)
➣ Management:
• Improved saturation with non-invasive ventilation (FiO2 50%).
• Steroids with nebulised bronchodilators.
• After symptom relief, the patient was released.
Protocol
● Physical Issues and Handling
• Pain, cramping in the muscles, exhaustion, and consequences from metabolic imbalances are prevalent in haemodialysis patients.
➣ Pain: Both acute and chronic pain are common side effects of procedures including vascular access operations and venipunctures. Regular pain assessment with standardised measures, pharmacological management (analgesics), and non-pharmacological approaches, such as relaxation and repositioning, are all part of nursing interventions.
➣ Muscle cramps: Electrolyte imbalances and fluid changes are frequently the cause of cramping. In order to control this, nurses make sure patients are properly hydrated both before and after sessions, carefully monitor fluid loss rates, and modify the composition of dialysate.
➣ tiredness: Chronic tiredness lowers life quality. Nursing interventions include working with dietitians to address nutritional deficiencies, promoting light physical activity as tolerated, and planning dialysis sessions to maximise rest.
● Metabolic and Treatment Difficulties
→ Issues such as uremic syndrome, toxin buildup, and dietary/fluid limitations make patient care more difficult.
➣ Dietary and Fluid Restrictions: Nurses regularly check for signs of malnutrition and fluid overload, promote adherence with monitoring, and educate patients on dietary recommendations and fluid allowances. Metabolic Management: The risks of uremic syndrome and toxin accumulation are reduced by routine blood chemistry monitoring, prompt medication modifications, and patient education.
Social and Psychological Problems
Psychological problems like social isolation, anxiety, and depression are linked to haemodialysis.
➣ Depression and Anxiety: Using standardised screening instruments, nurses routinely check for mood disorders, provide emotional support, and, if needed, coordinate social services or psychiatric referrals. It is frequently advised to promote involvement in therapy sessions and support groups.
● Nursing Interventions for Complications:
→ Patients receiving haemodialysis are susceptible to a number of complications, such as infections and problems with vascular access.
➣ Infection Control: Nurses use strict aseptic technique during access procedures, teach patients and carers about hygiene, and keep an eye out for infection symptoms.
➣ Vascular Access Management: Regular evaluations for patency and complications, timely reporting of abnormalities, and patient education regarding symptoms of access dysfunction are essential to avert serious consequences.
● Improving Quality of Life
→ It is essential for management to address economic, emotional, and therapeutic access concerns.
Socioeconomic Challenges: In order to assist patients in obtaining financial assistance or transportation for treatment, nurses collaborate with social workers.
➣ Adherence and Self-management: Results are greatly enhanced by offering individualised education, promoting participation in care planning, and scheduling frequent follow-up appointments to reaffirm adherence to dietary, lifestyle, and medical advice.
Notes
For more details visit 10.7759/cureus.40172
