Non-Specific Interstitial Pneumonia (NSIP)

Epidemiology

  • Mean Age of Onset: 50’s
  • Relationship to Smoking: unknown
  • NSIP pattern is usually idiopathic but may be seen in collagen-vascular disease, drug toxicity, and [[Hypersensitivity Pneumonitis]]

Etiology

  • Connective Tissue Disease
    • Scleroderma (see [[Scleroderma]]): NSIP is more common than UIP in scleroderma-associated ILD
    • Rheumatoid Arthritis (see [[Rheumatoid Arthritis]]): both UIP and NSIP are seen in RA-associated ILD
    • Sjogren’s Syndrome (see [[Sjogrens Syndrome]])
    • Polydermatomyositis (see [[Polydermatomyositis]])
  • Hypersensitivity Pneumonitis (see [[Hypersensitivity Pneumonitis]])
  • Diffuse Alveolar Damage
  • Infection
  • Lymphocytic Interstitial Pneumonia
  • HIV Disease (see [[HIV Disease]])
  • Infection
  • Chronic Eosinophilic Pneumonia
  • Idiopathic Pulmonary Hemosiderosis
  • Pulmonary Alveolar Proteinosis
  • Drugs
    • Cyclophosphamide (see [[Cyclophosphamide]])
  • Idiopathic Non-Specific Interstitial Pneumonitis

NSIP

(BAL lymphocytosis and/or resp failure are common)

  • Abacavir:
  • Anagrelide:
  • Aurothiopropanosulfonate (Gold Salt):
  • Azacitidine:
  • Beta Blockers:
  • Carbamazepine:
  • Celiprolol:
  • Cipro:
  • Cladribine:
  • Cytarabine (ARA-C):
  • Docetaxel:
  • Fenfluramine/Dexfenfluramine:
  • Fludarabine:
  • Imatinib:
  • Infliximab:
  • Inteferon Alpha:
  • Leflunomide:
  • Methotrexate:
  • Nilutamide:
  • Nitrofurantoin:
  • Procarbazine:
  • Rituximab:
  • Sulfamethoxazole+Trimethoprim (Bactrim, Septra):
  • Trastuzumab:
  • Trofosfamide:

Less Severe NSIP: BAL lymphcytosis, resp failure (less common)

  • Acebutolol:
  • Acyclovir:
  • Amiodarone:
  • Ampicillin:
  • Amrinone:
  • ACE-I: captopril
  • Antazoline:
  • Antidepressants: bepridil, maprotiline, metapramine, fluoxetine, venlafaxine
  • Atenolol:
  • Aurothiopropanosulfonate (Gold Salt):
  • Azapropazone:
  • Azathioprine:
  • BCG therapy:
  • Beta Blockers: carvediolol, nadolol, oxprenolol, practolol
  • Bicalutamide:
  • Bleomycin:
  • Bucillamine:
  • Carbamazepine:
  • Carmustine (BCNU):
  • Chlorambucil:
  • Clozapine:
  • Cromoglycate:
  • Cyclosporin:
  • Cyproterone Acetate:
  • Dasatinib:
  • Dihydralazine:
  • Dihydroergocryptine:
  • Disopyramide:
  • Docetaxel:
  • Dothiepin:
  • Ergot Alkaloids: ergotamine
  • Etanercept:
  • Etoposide:
  • Everolimus:
  • Flecainide:
  • Floxuridine:
  • Furazolidone:
  • GM-CSF:
  • Gemcitabine:
  • Glibenclamide:
  • Heroin:
  • Hydralazine:
  • Hydrochlorothiazide:
  • Hydroxyquinoleine:
  • Hydroxyurea:
  • Ifosfamide:
  • Irinotecan:
  • Lansoprazole:
  • Mefloquine:
  • Mercaptopurine:
  • Metformin:
  • Methysergide:
  • Misoprostol:
  • Mitomycin-C:
  • Mitoxantrone:
  • Nalidixic Acid:
  • Nitrosoureas:
  • Nomifensine:
  • Oxyphenbutazone:
  • Paclitaxel:
  • Penicillamine:
  • Phenytoin:
  • Piperacillin:
  • Pituitary Snuff:
  • Pranlukast:
  • Procainamide:
  • Propylthiouracil:
  • Pyrimethamine-Sulfadoxine:
  • Quinidine:
  • Raltitrexed:
  • Retinoic Acid:
  • Riluzole:
  • Simvastatin:
  • Sirolimus:
  • Sulfonamides: sulfasalazine
  • Temozolomide:
  • Temsirolimus:
  • Thalidomide:
  • Ticlopidine:
  • Tiopronin:
  • Tocainide:
  • Valsartan:
  • Vindesine:
  • XRT:

Pathology

  • Uniform and homogeneous temporal appearance
  • Prominent interstitial inflammation
  • Variable diffuse collagen fibrosis
  • Occasional diffuse or rare fibroblastic foci
  • Rare microscopic honeycombing
  • Occasional patchy intra-alveolar macrophage accumulation
  • No hyaline membranes

Diagnosis

  • HRCT: subpleural sparing

Clinical

  • Subacute or insidious onset

Treatment

  • Good response to steroids (complete recovery is possible)

Prognosis

  • 17-Year Mortality Rate: 11%