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VASCULITIS

DERMATOLOGY LECTURE NOTES

DEBABRATA BANDYOPADHYAY
PROFESSOR & HEAD, DEPT. OF DERMATOLOGY,
R. G. KAR MEDICAL COLLEGE, CALCUTTA, INDIA

 

 

The vasculitides are a heterogeneous group of clinicopathological entities that share the common feature of vascular inflammation and injury. There is no universally acceptable classification of these group of disorders. While a number of underlying causes can be identified in some disorders, the aetiology is unknown in many. The pathogenetic mechanisms involved are mainly immunological, immune complex mediated tissue injury being the most commonly incriminated factor.

 

 

ETIOLOGY & PATHOGENESIS

While some forms of vasculitides may be ascribed to underlying factors like infections, malignancy, drug reactions or connective tissue disorders, the cause may remain undetermined in many vasculitic syndromes. Immunologic damage by immune-complex deposition or cell-mediated hypersensitivity is responsible in the majority of cases.  

The possible immunopathologic mechanism in the causation of vasculitis are

1. Deposition of circulating antigen-antibody complex or in -situ formation of immune complex within the vessel wall. This leads to complement activation and chemotactic attraction of neutrophils by complement components. Subsequent phagocytosis of such complexes with liberation of neutrophil granular products leads to vascular damage.  

2. Cell-mediated hypersensitivity: Antigenic exposure may attract lymphocytes which liberate cytokines causing tissue damage and further activation of macrophages and lymphocytes. 

3. Failure to clear the antigen may lead to persistent inflammation and eventual formation of epithelioid cells and giant cells, giving rise to a granulomatous tissue reaction.  

Whatever the underlying mechanism, vascular inflammation and necrosis ensues which is often accompanied by thrombosis. These pathologic changes result in tissue ischaemia, necrosis and infarction, leading to a variety of clinical manifestations depending on the anatomic structures involved.

PATHOLOGY:

Perivascular cellular infiltration is a common histological finding in many disease entities, but for a definitive diagnosis of vasculitis, the presence of vascular damage, particularly in the form of fibrinoid degeneration, is necessary. Vasculitis may involve blood vessels of varying calibers and this feature forms the basis of a useful pathological classification of vasculitis. An infiltrate, composed of a variety of cell types, like neutrophils, lymphocytes, and histiocytes may invade the vessel wall and the surrounding tissue. Extravasation of red cells is a prominent feature in many vasculitides. Granulomatous inflammation with giant cell formation is a characteristic finding in some types. 

 

CLASSIFICATION

Vasculitis is a taxonomistís nightmare. Diseases with diverse causes and pathology may share the same symptomatology. On the other hand, a disease may show different histopathologic features at different periods in its evolution. Many diseases have overlapping features and it is impossible to formulate a classification scheme that unifies clinicopathological, etiological and immunological features of different diseases. 

          Immunopathologic classification:                   

                   I. Immune- complex mediated vasculitis:

                                                Polyarteritis nodosa
                                               
Microscopic polyangiitis
                                               
Hypersensitivity vasculitis
                                                (Leukocytoclastic vasculitis)
                                               
Henoch-Schonlein purpura 

                   II.  Vasculitis due to cellular hypersensitivity (Granulomatous
                         vasculitis)

                                                Giant cell arteritis
                                               
Takayasuís arteritis
                                               
Churg-Strauss disease (allergic
                                                                                granulomatosis)
                                               
Wegenerís granulomatosis
                                               
Isolated CNS vasculitis 

          Classification based on caliber of blood vessel involved:  

                   I. Large vessel vasculitis:
                                               
Giant cell arteritis
                                               
Takayasuís arteritis

                   II. Medium vessel vasculitis:
                                               
Polyarteritis nodosa

                                               
 Kawasaki disease
                  
III. Small vessel vasculitis:
                                               
Microscopic polyangiitis
                                               
Leukocytoclastic vasculitis
                                               
Wegenerís granulomatosis
                                               
Churg-Strauss disease 

          Classification based on cellular composition of the infiltrate:
                                          

I. Leukocytoclastic vasculitis (LCV): Neutrophils are predominant. Cellular fragments and nuclear debris (leukocytoclasia) are found in the infiltrate.                                                
                             
Polyarteritis nodosa
                              
Henoch-Schonlein purpura
                              
Vasculitis due to drugs, infections, and
                                    connective tissue diseases 
                              
Erythema elevatum diutinum
                              
Granuloma faciale etc. 

                   II. Lymphocytic vasculitis:
                                               
Lupus erythematosus
                                               
Lymphoma    
                                               
Pityriasis lichenoides

                   III. Eosinophilic vasculitis : 
                                               
Churg-Strauss vasculitis 

                   IV. Granulomatous vasculitis :
                                       
Wegenerís granulomatosis and Churg-Strauss 

                   V. Giant cell arteritis: 
                                     
Temporal arteritis
                                      
Takayasuís arteritis 

Clinical Classification:  

            I. Systemic necrotizing vasculitis : 

   Polyarteritis nodosa

         Classical

         Microscopic polyangiitis

   Allergic granulomatosis 

    Polyangiitis overlap    

                                                Wegenerís granulomatosis

          II. Giant cell vasculitis

                                      Temporal arteritis
                                               
Takayasuís disease
         

          III. Predominantly Cutaneous small vessel vasculitis :                   

                            A. Idiopathic 
                                  
   B. Secondary:
                                               

              • Infection: ( Streptococcus, Staph, TB, Leprosy, Hep.B and C, HIV, Subacute bacterial endocarditis, EBV, parvovirus, Rickettsia)
              • Drugs :Penicillin, sulpha, phenytoin, allopurinol, gold, thiazide, NSAIDs, Frusemide, quinidine, thiouracils, mefloquine
              • Connective tissue disease (SLE, Sjogrenís syndrome, RA, Scleroderma, Dermatomyositis )
              • Malignancy ( Lymphoma, leukemia, solid organ tumors )
              • Other diseases:

Cryoglobulinemia, complement deficiency, alpha1 antitrypsin deficiency  Inflammatory bowel disease, Chronic active hepatitis, intestinal bypass surgery, primary biliary cirrhosis ,Relapsing polychondritis  

                                            C. Clinical syndromes with leukocytoclastic
                                                vasculitis:                                                                    Henoch-Schonlein purpura
                                                                  
Urticarial vasculitis
                                                                  
Serum sickness
                                                                  
Erythema elevatum diutinum
                                                                  
Granuloma faciale
                                                                  
Hyperimmunoglobulinemia D
                                                                   Acute hemorrhagic edema
                                                                    of  children
                                                                  
Familial Mediterranean
                                                                     fever.
 

                   IV. Other vasculitic syndromes:                                                                    Behcetís disease                                                                    Buergerís disease
                                                                  
Kawasakiís disease
                                                                  
Isolated CNS vasculitis
                                                                  
Coganís syndrome

CLINICAL FEATURES

Features in an individual patient will depend on the specific vasculitic syndrome and the degree and extent of organ systems involved.            

                    Constitutional:  Fever, malaise, headache, weight loss etc 

                    Cutaneous

                                        Palpable purpura              Persistent urticaria

                                        Subcutaneous nodules     Ulcers

                                        Necrotic papule                Infarcts

                                        Hemorrhagic pustules 

                    Musculoskeletal:

                                        Arthritis                            Arthralgia

                                        Myalgia 

                    Renal            Proteinuria                        Hematuria

                                        Acute and chronic renal failure 

                    Respiratory:       Nasal obstruction, discharge, bleeding 

                                            Cough,        Hemoptysis        Dyspnea 

                    GI     :              Dysphagia      Pain  Vomiting     Bleeding 

                    CVS:                 Hypertension        Thrombosis    Ischemia 

                    Eyes:                Uveitis      Retinal vasculitis 

                    Nervous system:    Peripheral neuropathy  

                                            Stroke

For salient features of major vasculitic syndromes : CLICK HERE

 

 

LAB TESTS

 
 

1. Screening Tests:                             

                                CBC      ESR        CRP       ASO titre
                                Throat swab culture
                                Stool for OBT
                                Urinalysis
                                Chest skiagram
                                ECG
                                Renal screen
                                LFT
 

                    2. Focused Tests:                               

                                ANA                     C3, C4, CH50
                                Rh factor              Cryoglobulins

                           
APLA                    CK

                                ANCA screen         Serum protein electrophoresis
                               
                                Mantoux Test        HepB/C serology
                                Tests to detect other infections

                                EMG-NCV            Echocardiography
                                Sinus X-ray, CT scan
                                Angiography
 

                3. Biopsy of appropriate tissue:                

                                Skin                    Temproral artery

                                Kidney                Testes  etc

 

 
 

CLINICAL APPROACH

 
 

I. Suspect diagnosis:
                The following features should raise the suspicion of an underlying
vasculitic process:               

                Unexplained multisystem disease,       PUO
                CVA, particularly in young age           mononeuritis multiplex
                Persistent sinus symptoms                Glomerulonephritis
                Pneumonitis                                   GI bleeding
                Eye symptoms                                Arthritis/arthralgia
                Cutaneous signs:
                        Palpable purpura         Persistent urticaria    Subcutaneous nodules

                        Hemorrhagic pustules    Livedo reticularis       Infarcts

                        Digital gangrene 

II. History, Clinical exam and Lab investigations to detect general features of inflammation and  tissue ischemia and to delineate  organ systems involved.       

III. Syndrome recognition: recognize vasculitic syndromes based on clinical findings and lab features and the patterns of organ system involved. 

III. Confirmation of diagnosis: By biopsy and angiography 

IV. Exclusion of underlying cause like infections, connective tissue disorder or malignancy

 

 
 

MANAGEMENT

 
 

Treatment will depend on the specific syndromes, the degree and severity of organ system involvement and the underlying cause if any. 

1. Remove underlying cause: Withdraw drug, treat infection.

2. In mild cases no treatment or symptomatic measures like simple analgesics and rest will suffice.

3. Predominantly cutaneous vasculitis may respond to Dapsone, colchicine, hydroxycloroquine.

4. Systemic vasculitides or severe cutaneous vasculitis will require systemic steroids with or without immunosuppressive agents like cyclophosphamide, azathioprine or methotrexate. Cyclosporine may also be used in certain cases.

5. Symptomatic / specific measures for organ system failure.
 

 
 

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