Bharat kumar choudhary topic-Polyarthritis nodusa.pptx

Polyarthritis nodusa

1. DEPARTMENT OF INTERNAL MEDICINE AND RHEUMATOLOGY Topic:-Polyarthritis nodusa Professor:- Yuliya viktorovna petrova Bharat kumar choudhary Group-442(4 th course)

2. Definition Polyarteritis nodosa is a serious inflammatory blood vessel disease. The small and medium-sized arteries.It is a systemic vasculitis characterized by necrotizing inflammatory lesions that affect small and medium size muscular arteries. It’s become swollen and damaged.
Preferentially at vessel bifurcations, resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently organ ischemia or infarction.

3. ➡️Vasculitis and its consequences may be the primary or sole manifestation of a disease; alternatively, vasculitis may be a secondary component of another disease ➡️Vasculitis is a general term that refers to inflammation of the blood vessels. When inflamed, the blood vessel may become weakened and stretch in size, which can lead to aneurysms, or become so thin that they rupture resulting in bleeding into the tissue. Vasculitis can also cause blood vessel narrowing to the point of closing off entirely so that organs become damaged from loss of oxygen and nutrients that were being supplied by the blood.
PAN exclusively affects medium-sized blood vessels.

4. Classification of vasculitis:- #LARGE SIZED VESSELS
1)Temporal arteritis
2) Takayasu’s arteritis #MEDIUM SIZED VESSELS
1) Polyarteritis nodosa 2)Kawasaki’s disease

#SMALL SIZED VESSELS
1)Wegener’s granulematosis 2) Churg -Strauss syndrome 3)Microscopic polyangiitis

5. History of disease Kussmaul and Maier first described PAN in 1866. The autopsy of a patient with fever, weight loss, abdominal pain, and polyneuropathy revealed areas of focal inflammatory exudations that gave rise to palpable nodules along the course of medium-sized arteries

6. Epidemiology:- Polyarteritis nodosa (PAN) is a rare disease with an incidence of about 3-4.5 cases per 100,000 population annually.
Sex/Gender:-PAN affects men more frequently than women (male-to-female ratio 1.6-2:1).
Age:-it is predominantly observed in individuals aged approximately 45-65 years.

7. Pathophysiology:- Vascular lesions in medium-sized muscular arteries occur main. Bifurcations and branch points.
Inflammation may start in the vessel intima and progress to include the entire arterial wall, destroying the internal and external elastic lamina, resulting in fibrinoid necrosis.
Aneurysms develop in the weakened vessel, carrying a subsequent risk for rupture and hemorrhage.
Thrombi may develop at the site of the lesions
As lesions progress, proliferation of the intima or media may result in obstruction and subsequent tissue ischemia or infarction.
It spares large vessels (the aorta and its major branches), the smallest vessels (capillaries and small arterioles), and the venous system.

8. Causes/Etiology 1)The cause of PAN is unknown(idiopathic).
2)Immune system plays a critical role in PAN
3)PAN is highly associated with hepatitis B virus infection- {HBV was once the cause of up to 30% of PAN cases}Evidence for immune complex-induced disease is confined to HBV-related PAN, the role of immune complexes in non-HBV-related PAN remains unclear.
4)Impaired function of endothelial cells may be part of idiopathic PAN or a consequence of it in HBV-PAN, virus replication may directly injure the vessel wall.
5)Endothelial dysfunction can perpetuate the inflammation through cytokine and adhesion molecule production.

9. Sign & Symptoms:- Polyarteritis nodosa (PAN) is an acute multisystem disease with a relatively short prodrome (weeks to months).
The spectrum of disease ranges from single-organ involvement to fulminant polyvisceral failure .
Most common symptom includs :-
Fever
Malaise
Fatigue

10. Abdominal pain
Loss of appetite Anonesia and weight loss
Myalgia(muscle pain)
Arthralgia in large joints
Less commonly-arthritis
skin sores( hard tender nodules or ulcers) Hematochezia as a result of GIT involvement
shortness of breath or chest pain
High blood pressure is common in PAN and usually due to vasculitis decreasing blood flow to the kidneys. PAN may affect nerves and cause abnormal sensations, numbness or loss of strength.

11. Renal Symptoms About 50% of patients with PAN have renal involvement.
Flank pain
Ischemic changes in the glomeruli and renal artery vasculitis can cause renal failure.
A small percentage of patients may require dialysis.
Renal symptoms including:-
Hypertension Costophrenic tenderness
Retroperitoneal or intraperitoneal hemorrhage.
Renal failure

12. Cutaneous symptoms Dermatologic symptoms are very common in PAN, and about 40% of patients manifest with skin lesions including:-
Rash, Purpura Gangrene
Nodules
Cutaneous infarcts livido reticularis Raynaud phenomenon.
Skin involvement, which can be painful, occurs most frequently on the legs.

13. #Central nervous system symptoms:- Monocular blindness,
Arterial thrombosis with cerebral ischemia or intra-parenchymal or subarachnoid hemorrhage.
CNS dysfunction with encephalopathy and seizures
Multiple organ failure.
Acute or subacute myelopathy. #Peripheral nervous system symptoms:- Peripheral neuropathy develops in as many as 60% of patients. Vasculitic neuropathy is often asymmetrical and presents as
(1) mononeuritis multiplex
(2) distal polyneuropathy
(3) cutaneous neuropathy.
It can take the form of a pure motor, pure sensory, or mixed sensorimotor polyneuropathy

14. #Gastrointestinal symptoms:- GI involvement usually presents as nonspecific symptoms and signs such as:-
Abdominal pain,
Nausea and vomiting with or without obvious GI bleeding.
Rare and more serious complications of PAN include:-
Bowel infarction and perforation,Cholecystitis,Hepatic infarction,Pancreatic infarction. #Neurological symptoms:- Sensory and motor neuropathies occur, they are usually asymmetrical.
Multiple mononeuropathy ischemia or infarction, ulnar, radial, peroneal, sural nerve
CNS involvement –encephalopathy, focal deficits, strokes, seizures and, brain hemorrhages.

15. Less common symptom #Genitourinary symptoms:-P atients may develop pain over the testicular or ovarian area. In rare cases, testicular infarction may occur, testicular pain is usually unilateral #Cardiac symptoms:- Chest pain, dyspnea, palpitations, pericarditis, myocardial infarction,hypertension,tachycardia Pericardial friction rub,arrhythmias Congestive heart failure
cardiac disease affects 35% of patients with PAN, but most affected patients are asymptomatic #Ophthalmologic symptoms :- Blurred vision
Retinal vasculitis
Retinal detachment
Cotton-wool spots #Neuropsychiatric symptoms:- Headache, psychosis, and depression

16. Genetic association:- Loss-of-function mutations in CECRI, the gene that encodes adenosine deaminase 2 (ADA2), have been associated with a spectrum of vascular and inflammatory phenotypes that includes polyarteritis nodosa Possible roles of ADA2 include regulation of the proliferation of activated T cells and macrophages and the differentiation of monocytes to macrophages. Reduction in ADA2 activity may affect the adenosine inflammatory-response pathway.

17. Polyarteritis Nodosa Association with Hepatitis B (surface antigen)
Classic PAN is NOT associated with ANCA
the potential association of hepatitis C virus (HCV) with PAN.
INFECTIOUS organisms include varicella-zoster virus,parvovirus 8-19,cytomegalovirus
human T-cell leukemia virus,
Streptococcus species, Klebsiella species, Pseudomonas species, Yersinia species, Toxoplasma gondii , Rickettsiae , trichinosis, and sarcosporidiosis Recently, reports of associations with PAN and human immunodeficiency virus and cutaneous PAN and tuberculosised to cutaneous PAN.. A benign, limited form of PAN
Rheumatoid arthritis and Sjögren syndrome have been associated with PAN Hematologic malignancies, such as hairy cell leukemia and angioimmunoblastic T cell lymphoma, have been associated with PAN-like vasculitides .

18. ACR Criteria (3 of 10) 1)Weight loss > 4 kg
2) Livedo reticularis 3)Testicular pain
4) Myalgias , weakness or leg tenderness
5) Mononeuropathy or polyneuropathy
6)Diastolic BP > 90
7)BUN or Creatinine
8)Hepatitis B virus
9) Arteriographic abnormality
10)Biopsy of small or medium artery containing PAN

19. Diagnosis Elevated ESR and CRP
Leukocytosis,
Normochromic anemia, Thrombocytosis
Elevated creatinine level
Mild proteinuria
Elevated levels of liver enzymes
Hyper- gammaglobulinemia – Found in 30% of patients with PAN Cryoglobulins , circulating immune complexes, and decreased levels of serum complement (ex. C3, C4) may be observed in patients with HBV-related PAN but are otherwise uncharacteristic of idiopathic PAN Instrumental:x-ray,ct-scan,mri

20. Treatment ➡️Immunosuppression continues to be the standard therapy for polyarteritis nodosa (PAN).
➡️Corticosteroids plus cyclophosphamide (in the case of steroid-refractory disease or major organ involvement) can prolong survival for patients with idiopathic PAN
➡️In contrast, for hepatitis B-related PAN, treatment consists of
corticosteroids for early, initial control followed by plasmapheresis and antiviral agents.
➡️Stronger immunosuppression using a combinations of steroids and cyclophosphamide is typically avoided in these cases as it can enhance viral replication.

21. Reference:- 1. Radiopedia Polyarteritis nodosa Available:https :// radiopaedia.org /a rticles /polyarteritis-nodosa-1 (accessed 23.1.2023) 2. Manual: polyarteritis nodosa . http://www.merckmanuals.com/ho me/sec05/ch069/ch069b.html (accessed 22 Feb 2011). 3. Cedars-Sinai polyarthritis nodosa . http://www.cedars- sinai.edu /Patients/Health- Conditions/ Polyarteritis - Nodosa.aspx (accessed 22 Feb 2011). 4. ↑ Goodman CC, Fuller KS.
Pathology: Implications for the Physical Therapist. 3rd edition. St. Louis, Missouri: Saunders Elsevier, 2009. 5. ↑ Kote's Medical Animations. Polyarteritis nodosa made easy.
Available from: https://www.youtube.com/watch? v=YrOejvJ2WyI [last accessed 23.1.2023]